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pmc-6232614-1
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A 54 year old male, with no comorbidities or significant social history presented to the emergency department with a three day history of intermittent generalized abdominal pain radiating to the back. He did not endorse a history of constitutional symptoms. Upon presentation to the emergency department he was hemodynamically stable. Physical examination revealed a soft abdomen with generalized tenderness particularly in the epigastric region. Initial lab results showed a WBC 6.77 × 109/L, serum amylase 79IU/L, urinary amylase 738IU/L, CRP 73.88 mg/L, D-dimer 13.66ug/ml, and a Lactate of 1.87 mmol/L. The patient was subsequently admitted under the gastroenterology service with the diagnosis of pancreatitis.
Preliminary CT Abdomen with oral and IV contrast showed acute pancreatitis with superior mesenteric and portal vein thrombosis and no evidence of mesenteric small bowel involvement (). He was managed conservatively on the ward by means of heparin Infusion and thereafter switched to LMWH and warfarin. A week later he developed sustained tachycardia and sudden drop in WBC with associated increase in lactate to 5.31IU/L. A follow up CT abdomen with IV contrast revealed diffuse wall thickening involving multiple loops of jejunum highly suspicious of bowel ischemia (). A decision was made to proceed with an exploratory laparotomy which revealed no evidence of full thickness necrosis but characteristics of small bowel edema secondary to SMV thrombosis. Additionally, the pancreas appeared inflamed and edematous with evidence of intraperitoneal calcification within the lesser sac. An ABthera vacuum dressing was applied in anticipation of a second look laparotomy. The patient was shifted to the ICU where he showed evidence of progressive acute hepatic failure with rising INR, bilirubin, and serum ammonia level. On re-evaluation of the bowel within 48 h, we noted viable but dusky appearing jejunum. An access sheath was inserted retrograde from the middle colic vein into the superior mesenteric vein and portal vein where a catheter was placed for the direct thrombolysis (). A bolus of Heparin 1000 IU followed by 500 IU/hr infusion was delivered through the sheath along with Altepase 1 mg/hr for a 24 h period.
Following the 24 h period, a third look exploratory laparotomy was performed where the vascular catheter placed was removed. An Intraoperative venography showed patent portal veins (). However, 100 cm segment of proximal bowel was found ischemic and therefore a resection and side to side anastomosis was done.
The patient had an uneventful post-operative course. A week after his final laparotomy, an ultrasound abdomen was performed showing resolved portal and superior mesenteric vein thrombosis. The patient was discharged in a satisfactory condition on Warfarin 3 mg PO OD.
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pmc-6232616-1
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A 67-year-old male patient with no significant medical history presented to our institution to check a mediastinal enlargement incidentally found on routine chest X-ray. Echocardiographic examination revealed a solid mass surrounding the right cardiac chambers, and computed tomography of the chest confirmed the presence of a right lateralized 12 × 4 cm soft tissue mass beginning in the antero-superior pericardium recess down to the right atrium and right ventricle (). The mass did not contain calcification and it appeared adjacent with the right atrium. There was no pericardial effusion.
The subsequent techniques included a completely unremarkable coronariography with no signs of any neovascularization to the mass. The magnetic resonance imaging (MRI) confirmed the presence of an intrapericardial mass, with hypersignal in T2, localized in the anterior and superior pericardial recess, with inferior extension along the interatrial groove, and free wall of the right atrium and ventricle, surrounding the right coronary artery, but with apparent cleavage plane ().
Median sternotomy approach was used to access the mass. The mass was completely adherent to the right atrium, right ventricle, and right coronary artery (). Due to this adherence, and the lack of a pathologic diagnosis, we ruled it unsafe to attempt a total resection of the mass, and instead performed a partial resection. The macroscopic examination of the cut surface revealed a large cystic space, with smaller spaces dispersed in a fibrotic wall ().
The patient had a normal post operative recovery and was discharged four days after the surgery. The patient was clinically well after one month.
The pathology specimen showed a mass containing lymphoid tissue, and the immunohistochemistry stains were consistent with a cardic lymphangioma.
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pmc-6232618-1
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An 83-year-old man was admitted to our hospital for evaluation and management of a symptomatic liver mass. His medical history included diffuse large B-cell lymphoma, which was treated with rituximab + pirarubicin + cyclophosphamide + vincristine + prednisone therapy at 81 years old, and had bladder cancer (resected at 67 years) on follow-up. After resection of the bladder cancer, no recurrence was detected for 16 years. Liver dynamic computed tomography (CT) showed a low-density mass in the segment (S) 4 area, measured 40 mm in diameter. The density of the tumor was well enhanced in the arterial phase and washed-out in the portal phase. (a–d). The hepatobiliary phase of Gd-EOB-DTPA-MRI shows tumor nodules in the liver with low intensity (e). On positron emission tomography (PET)-CT, the maximum standard uptake value of the tumor in S4 of the liver was 3.2 (f). MRI and PET-CT confirmed a single liver tumor that was 40 mm in diameter and located in the S4 region. Liver metastasis of malignant lymphoma was suspected because of the patient’s medical history. Therefore, we performed a liver biopsy preoperatively. The patient was diagnosed with hepatocellular carcinoma (HCC) based on the biopsy results and imaging findings.
Upon presentation, the patient was afebrile, had no history of weight loss, and his appetite was good. His height was 166 cm, body weight 72 kg, and BMI 26.12. He has no drinking history. In a preoperative indocyanine green (ICG) test, the ICGR15 was 76.2%. The total bilirubin level was 1.1 mg/dL and the direct bilirubin level was 0.2 mg/dL. The serum albumin level was 4.7 g/dL and prothrombin activity was 96.3%. The Child–Pugh (CP) score was 5 points, which indicated a grade of A. The degree of liver damage was equivalent to A in accordance with the scoring system of the Liver Cancer Study Group of Japan. shows the patient’s laboratory data on admission. The hepatic uptake ratio of 99mTc-galactosyl human serum albumin (GSA) by liver scintigraphy (LHL15) was 0.931 and the heart uptake ratio (HH15) was 0.482. The maximal removal rate of 99mTc-GSA (GSA-Rmax) was 0.874 mg/min. GSA-Rmax in the predicted residual liver (GSA-RL) was greater than 0.765 mg/min, which was within the range considered safe for surgical procedures.
Despite this finding, Child–Pugh classification and 99mTc-GSA liver scintigraphy did not show any abnormal findings, and there was no background disease. Antibody against hepatitis C virus and hepatitis B virus surface antigen were negative. The serum anti-mitochondrial antibody and anti-nuclear antibody were negative. The serum tumor markers alpha-fetoprotein, carcinoembryonic antigen, and cancer antigen 19-9 were within the and normal range, but the protein level induced by vitamin K absence-II levels was increased (92 mg/dL). Therefore, we diagnosed constitutional ICG excretory defect with HCC and decided to perform radical surgery. Therefore, the patient underwent partial hepatectomy (S4). Pathologically, the tumor was diagnosed as moderately differentiated HCC (a). There was expansion and bleeding of perisinusoidal cells and an atrophic hepatic cord in the background of liver tissue. Because of previous chemotherapy, the diagnosis of sinusoidal obstruction syndrome (SOS) of the liver was established (b). After partial hepatectomy (S4), the postoperative course was uneventful and the patient was discharged on the 8th postoperative day. The patient remains in good general condition.
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pmc-6232619-1
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An 86 year old man was referred for consultation in the surgical outpatients clinic regarding investigation of a 5 month history of abdominal pain, weight loss, nausea and diarrhoea.
He had a CT scan prior to referral demonstrating a small amount of ascitic fluid.
The patient had no past medical history of note. His last endoscopy and colonoscopy were 10 years ago, with the colonoscopy identifying a tubular adenoma and hyperplastic polyps in rectosigmoid region.
On review he had observations within normal parameters and his abdominal examination was normal.
A subsequent endoscopy revealed diffuse gastritis with a small antral gastric ulcer with a small amount of blood. Helicobacter pylori testing was negative. On colonoscopy there was moderate sigmoid diverticular disease. Three polyps were removed from the ascending colon, sigmoid colon and rectum. The patient was commenced of a proton pump inhibitor and review in rooms arranged for 4 weeks.
On review at 4 weeks the patient reported a complete resolution of symptoms that he had prior to initial consultation. His only complaint was of slightly more flatus than usual.
Abdominal examination was again unremarkable. A follow up endoscopy was arranged which was normal.
A routine abdominal CT scan was arranged to assess the presence of residual free fluid that was seen on the CT scan performed prior to the patient’s referral.
The CT scan demonstrated free air within the bowel wall, with a follow up scan performed 3 weeks later revealing an increase in the amount of free air. The patient had a barium swallow, which was negative for a leak.
The patient was referred to a tertiary centre for management, hyperbaric therapy was considered but ultimately the patient was treated conservatively with oral oxygen therapy and antibiotics.
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pmc-6232627-1
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A 20 year-old woman with no relevant past medical or surgical history, recurred to the emergency department of our institute because of left flank pain and fever. On physical examination tenderness at percussion of left lumbar region was observed, the pulse was 90 beats per minute, and the blood pressure 115/75 mmHg. The with-cell count was 14,200 per cubic millimeter, the plasmatic creatinine concentration was 0,9 mg per deciliter and urinalysis was positive for nitrites. The patient was discharged with the diagnosis of uncomplicated left Pyelonephritis and treated with a 7-day regimen of levofloxacine. The patient was completely asymptomatic after completing the treatment, however in the next 12 months she developed 10 episodes of recurrent non-complicated left-sided Pyelonephritis. In all the episodes a urine culture revealed more than 10,000 colony-forming units of Escherichia coli per milliliter of urine. The US examination of kidney and bladder revealed no alterations and the contrast enhanced CT scan performed at emergency in one of the episodes revealed a heterogeneous uptake of intravenous contrast in left kidney in favor of pyelonephritis, but absence of urinary system obstruction (, ).
The patient was referred for our Urologic Department for evaluation of recurrent Pyelonephritis and a prophylactic antibiotic regimen of nitrofurantoin 100 mg once a day was prescribed. The urologic evaluation was completed with a renal DMSA scan and VCUG. Renal DMSA scan revealed a left kidney with decreased uptake of DMSA with several cortical lesions. The differential kidney function was 70% for right kidney and 30% for the left kidney () and the VCUG revealed a left grade II VUR (). The patient was submitted to endoscopic treatment of left sided VUR with subureteric injection of dextranomer/hyaluronic acid copolymer (Deflux®). The procedure was uneventful and post-operative VCUG revealed complete resolution of VUR. After 6 months of endoscopic treatment the patient is completely asymptomatic without any report of Pyelonephritis.
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pmc-6232694-1
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A 29-year-old healthy African American female presented to the emergency department complaining of a 1-day history of peri-umbilical pain migrating to the right lower abdominal quadrant with associated anorexia, nausea, and vomiting. She had an onset of menses the day prior to onset of abdominal pain. On physical exam, the abdomen was soft, non-distended, but tender to palpation over McBurney’s point. Vital signs were within normal limits without notable fever or tachycardia. Blood work revealed an elevated white blood count of 17.4 K/UL. Alvarado score was calculated to be 9. CT of the abdomen with IV contrast exhibited no evidence of acute intra-abdominal or intra-pelvic process. Ultrasound of the pelvis disclosed dilated non-compressible distal appendix suggestive of appendicitis.
Diagnostic laparoscopy was performed which found 30 cc of blood in the pelvis attributed to a ruptured 3 cm left hemorrhagic ovarian cyst. The appendix appeared unusually contracted upon itself without evidence of erythema or surrounding acute inflammation. No peritoneal studding or endometrial implants were identified on laparoscopic evaluation of the abdomen or pelvis, and the omentum was not found in the right lower quadrant. She recovered uneventfully from her operation, and in follow-up her pre-operative pain had disappeared.
Microscopic examination of the appendix showed no pathologic evidence for acute appendicitis. The appendiceal lumen was lined by normal-appearing appendiceal mucosa (), and the serosa showed no polymorphonuclear cells but did show collections of benign endometrial-type glands and stroma, consistent with endometriosis (Red arrow, right). (H&E, 40× magnification) In , higher power view showed benign endometrial-type glands and stroma. (H&E 100× magnification)
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pmc-6232707-1
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A 59-year-old Caucasian man with a history of severe mitral regurgitation and recent diagnosis of seropositive RA was admitted to the hospital for evaluation of refractory joint pain and swelling. Four years prior to admission, the patient had undergone prosthetic mitral valve replacement. Since then, he had received deep dental cleanings twice a year. The patient was in his usual health until 11 months prior to admission, when he developed intermittent pain and swelling of his knees, right hip, right elbow, and wrists bilaterally that was associated with morning stiffness of >1 h. He endorsed 11 lbs. weight loss and night sweats, but no fevers. Following 6 months of persistent symptoms, the patient saw a local rheumatologist who noted synovitis of the 2nd left metacarpophalangeal joint and tenosynovitis of the extensor tendons of his left hand. Laboratory workup showed evidence of systemic inflammation [C-reactive protein (CRP) 100 mg/L, erythrocyte sedimentation rate (ESR) 84 mm/h] and positive ACPAs (measured by the anti-CCP antibody assay). Testing for RF was negative. The patient was diagnosed with early seropositive RA, and he was started on immunosuppression with prednisolone and methotrexate. Given lack of clinical improvement, leflunomide was added. Due to persistent joint pain and swelling, the patient was hospitalized 2 months later for evaluation.
At the time of hospital admission, laboratory evaluation showed CRP 112 mg/L, ESR 79 mm/h, and high-titer anti-CCP IgG antibodies (262 U/mL; reference range <17 U/mL). Musculoskeletal ultrasound (US) showed effusion of the 2nd and 3rd right proximal interphalangeal joints as well as 1st and 4th right metatarsophalangeal joints. There was evidence of tenosynovitis of the right wrist extensor tendons, and inflammation of the flexor tendons of the right ankle and right Achilles tendon. Radiographs of the hands and feet showed no erosions. Prednisolone was increased. The patient was started on etanercept, and leflunomide was discontinued. Following a brief period of improvement, the pain around the right Achilles tendon and right wrist flexor tendons worsened within 3 weeks. US revealed new abscess formation along the right Achilles tendon. Incision and drainage was performed with wound cultures demonstrating Aa by PCR and sequence analysis. Blood cultures grew Aa in 2/3 sets of bottles, and echocardiography confirmed prosthetic mitral valve endocarditis. All immunosuppressive medications were discontinued, and antibiotic therapy with ceftriaxone 2 g IV daily was started. CRP levels decreased, and the joint pain improved. After completing a 6 week course of intravenous antibiotics, the patient's joint pain and swelling had resolved. Thereafter, anti-CCP antibody levels started to decline (Figure ). A non-ulcerated squamous cell carcinoma of the tongue was subsequently diagnosed and treated with radiotherapy. At ~1 year follow-up, the patient remained free of joint symptoms, and anti-CCP antibodies and CRP levels had normalized (anti-CCP 13.5 U/mL; CRP 5 mg/L) (Figure ).
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pmc-6232774-1
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A 59-years-old woman presented with sudden onset of lethargy, slurred speech, and left extremity weakness since 5 h. Neurological examination indicated right gaze preference, dysarthria, and decreased muscle strength on the left side (grade II). The patient had a NIHSS score of 8. Previously, the patient presented with paroxysmal dizziness for 1 year and had no history of brain trauma. No intravenous tissue plasminogen activator (tPA) was given since symptom onset was 5 h after presentation to the emergency room.
The patient was admitted and transferred to the catheter room 5.5 h after the onset of symptoms. Digital subtraction angiography (DSA) demonstrated a filling defect caused by a long segment severe stenosis in the BA, which was first assumed to be intraluminal clot related to BA stenosis. After a 6Fr guiding catheter (Envoy, Cordis) was placed into the right vertebral artery (VA), a microcatheter (REBAR-21, Covidien) co-axially assembled with a 0.014-inch Synchro Standard microwire (Stryker, Neurovascular) was used to traverse through the lesion. A self-expanding stent retriever (SOLITAIRE AB 6–30 mm, Covidien) was deployed across the lesion. Mechanical thrombectomy (MT) was performed; however, no clot was found. Repeat DSA showed even worse antegrade flow. It was decided to deploy the stent retriever which lead to restored caliber of the BA. IAD rather than ICAS was suspected. Nevertheless, conventional DSA failed to confirm the diagnosis of IAD. After the procedure, the patient regained consciousness and speech without gaze preference. The muscle strength on the left side recovered to grade III. Intravenous platelet glycoprotein IIb/IIIa receptor inhibitors (Tirofiban, Yuanda Pharmaceuticals, Wuhan, China) was maintained (5 ml/h) for 18 h after the procedure. Double anti-platelet regimen (aspirin 100 mg plus clopidogrel 75 mg per day) was given for 3 months (aspirin 100 mg alone thereafter). Post-operative Diffusion-weighted imaging (DWI) showed acute infarctions in the right pons and occipital lobe (Figure ). The patient had a NIHSS score of 2 at discharge and 0 at 3-months follow-up, respectively. The modified Ranking Score at 3 months was 1.
The patient had no recurrent symptoms until 18-months follow-up. She was transferred to our institute due to paroxysmal dizziness and blurred vision for the past month. In-sent restenosis was confirmed on follow-up angiography (85% based on WASID criteria) (). The restenosis was located within the proximal tapered area of the SOLITAIRE stent (Figure ).
The need for further intervention of this restenosis was uncertain based on DSA alone. Therefore, OCT was performed in order to assess the underlying cause of restenosis and confirm the diagnosis of IAD. The patient has signed informed consent regarding the use of OCT which was approved by the local Institutional Review Board. The intravascular frequency-domain OCT system (ILUMIEN OPTIS, OCT Intravascular Imaging System; St. Jude Medical) was used. After conventional angiography, the patient was placed under general anesthesia. A bolus of 5,000 units of intravenous heparin was administrated. With a 6Fr intermediate catheter (NAVIEN 115 cm long, Covidien) placed in the right VA, a 0.014 inch 300 cm long microwire (PILOT 150, Abbott) co-axially assembled with a microcatheter (ECHELON-10, Covidien) was carefully advanced through the proximal stent marker, the area of restenosis, and placed in the right posterior cerebral artery (PCA). After that, the microcatheter was exchanged for a 2.7Fr OCT imaging catheter (Dragonfly Duo; LightLab Imaging, Inc., St. Jude Medical).The short “monorail” design of the Dragonfly catheter did not permit its proximal marker to enter the PCA despite many attempts. After the catheter was advanced as far as in the mid-BA, control angiography demonstrated the opacification of the BA dissection.
Imaging at multiple levels was performed along the BA with an automatic pullback speed (36 mm/s) during blood clearance by the injection of contrast medium. The OCT data were analyzed by the ILUMIEN OPTIS Imaging System. OCT imaging demonstrated visualization of a dissection and poor stent strut wall apposition (Figure ). The intimal disruption was limited to the VA and the false lumen extended into the BA. There were no clot formation or tissue prolapse within the stent. After the OCT imaging catheter was withdrawn, control angiography demonstrated rapid antegrade flow and improved lumen at the site of the previously demonstrated restenosis. No progressive stenosis or occlusion was noted after 10 min observation and no additional intervention was needed. The patient was given intravenous Tirofiban for 24 h after procedure. She had no symptoms and was discharged without neurological deficits 3 days after the procedure.
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pmc-6232786-1
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A 1415-gram female infant was delivered at 346/7 weeks of gestation to a 40-year-old primigravida by cesarean section secondary to preeclampsia and abnormal middle cerebral artery Doppler assessment. Apgar scores were 8 and 9 at 1 and 5 minutes, respectively. Pregnancy was complicated with a diagnosis of severe intrauterine growth retardation. Family history revealed a 7-year-old half-sister with hereditary anemia. There was no in utero exposure to known teratogens. No genetic test was performed during pregnancy. Physical examination revealed a weight of 1415 g (<3rd centile), length 34 cm (<3rd centile), and head circumference 29 cm (5th centile). He was noted to have downslanted palpebral fissures, low-set and posteriorly rotated ears, wide space nipples, palmar crease, small hands and feet, rocker bottom feet, overgrowth 2nd toes, and overlapping 3rd and 4th toes (Figures , , , , and ). Chest radiography revealed 11 ribs. Cranial MRI scan showed mildly dilated lateral and third ventricles, and there was a 17x13 mm arachnoid cyst at the velum interpositum (). Echocardiogram revealed left-side aortic arch. Genetic testing was performed at 4 days of age. During 4 weeks of hospitalization, asymmetrical growth of left and right sides of the body and extremities was noted (). The infant's blood count and red cell indices (mean corpuscular volume) were unremarkable. She was discharged home at 31 days of age.
On the blood sample that was collected for genetic testing on day 4 of life, whole genome SNP (Single Nucleotide Polymorphisms) microarray analysis was performed using the Affymetrix CytoScan HD platform which uses over 743,000 SNP probes and 1,953,000 NPCN probes with median spacing of 0.88 kb. 250 ng of total genomic DNA extracted from lymphocytes was digested with NspI and then ligated to NspI adaptors, respectively, and amplified using Titanium Taq with a GeneAmp PCR system 9700. There was a 77 kilobase (kb) microdeletion at 11p15.4 arr [hg19] 11p15.4(5,191,871-5,268,465) x 1(). The deleted region includes 3 OMIM genes (HBB, HBD, and BGLT3).
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pmc-6232787-1
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A 21-year-old female of Maltese ethnicity, without a family history of IED, was diagnosed with IED following a Ladd procedure for intestinal malrotation at the age of three months. This was confirmed on open jejunal biopsies. Her medical treatment thus pursued shortly after this period with total parenteral nutrition (TPN) and oral and intravenous steroids. The latter had accounted for her short stature. Despite this, she led a normal life and was independently mobile and pain free up until the age of 18. She remained independently mobile until the age of 19 when she developed bilateral hip and knee arthritis.
Clinically, features were consistent with acute inflammatory polyarthropathy which were confirmed on plain radiographs (Figures and ) and serial MRIs (Figures and ). Her initial physical examination revealed marked knee effusions. Blood investigations included inflammatory markers—erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP), blood count, liver function, rheumatoid factor, antinuclear antibody (ANA), and anticyclic citrullinated peptide (anti-CCP). All results were within the normal accepted range values.
In view of clinical and radiological evidence of synovitis, she was treated with methotrexate followed by infliximab. Despite such treatment, as well as several pulses of intravenous steroids, the disease progressed rapidly within six months by which time her hips were almost fused in fixed flexion/abduction and her knees were fixed in 30-degree flexion.
Initial MRI of the hips (at age 19) showed bilateral symmetrical concentric loss of joint space with areas of full-thickness chondral loss and associated subchondral cystic change in relation to either hip joint. There are small associated hip joint effusions. Overall appearances would point towards a low-grade inflammatory arthropathy, rather than primary degenerative changes.
Follow-up MRI on the hips (age 20) showed bilateral established hip articular degenerative changes with associated hip joint effusions and synovitis. No avascular necrosis pattern was being demonstrated, with bilateral femoral head small focal erosive changes.
She was referred to the Nuffield Hospital Orthopaedic Centre in Oxford, UK, where she underwent simultaneous hip arthroplasties initially (), followed by a unilateral knee arthroplasty () one month proceeding. Her bone quality was found to be osteopenic more so at the distal femur compared to around the proximal femur and acetabulum. Histology of the femoral heads () was confirmed as inflammatory arthropathy with late degenerative changes.
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pmc-6232788-1
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A 49-year-old, premenopausal, asymptomatic woman, with past clinical history significant for total hysterectomy 10 years earlier due to a leiomyoma of the uterus, presented with a miliary pattern in a routine chest radiography as in computed tomography (CT) scan (). We performed a Positron Emission Tomography (PET) scan that showed weak fluorodeoxyglucose (FDG) uptake in lung nodules. She underwent CT-guided biopsy of a pulmonary nodule which revealed spindle cells consistent with smooth muscle differentiation, without cellular atypia, necrosis, or mitotic figures. Immunohistochemical examination was positive for smooth muscle actin (SMA), desmin, estrogen, and progesterone receptors and was negative for HBM-45, CK7, and S100. The proliferative index, assessed with Ki-67 index, was low. Cytogenetic evaluation of lung tumor tissue showed 19q and 22q terminal deletions. Cytogenetic analysis of previous leiomyoma was not performed due to insufficient pathological material. After diagnosing BML, patient underwent bilateral salpingo-oophorectomy followed by Letrozole therapy. At 9 months follow-up, there was no further development of the disease.
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pmc-6232788-2
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A 48-year-old premenopausal woman was referred because of persistent cough. Her past clinical history included a hysterectomy 13 years earlier for uterine leiomyoma. Chest radiography and CT revealed multiple pulmonary bilateral nodules () with no FDG uptake in the PET scan. CT-guided biopsy of a pulmonary nodule was performed and the resected uterine leiomyoma was reviewed. Both specimens showed identical histopathology of a low grade, benign appearing, and smooth muscle tumor (). The immunohistochemical profile of BML is indistinguishable from that of the primary uterine tumor with positivity for SMA, desmin, estrogen, and progesterone receptors () and negativity for HMB-45, CD31, CD34, and EMA. The staining for ki-67 showed low mitotic activity. Cytogenetic analysis revealed shared profile between both samples, including 19q and 22q terminal deletions (). Since these findings were consistent with BML, surgical castration was performed. After 6 months of follow-up, the remaining lesions were stable.
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pmc-6232790-1
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A 78-year-old man with heart failure and low ejection fraction was referred to our institution. Electrocardiogram showed sinus rhythm, heart rate of 82 beats, and complete left branch bundle block. Laboratory data indicated 1.04 mg/dL of creatinine, 7.0% of HbA1c, and 268 pg/mL of brain natriuretic protein. Echocardiography showed an ejection fraction of 30%, left ventricle diastolic diameter of 60 mm, diffuse hypokinesis, and apical akinesis. Angiography after compensated heart failure revealed hypoplasty of the right coronary artery, severe stenosis with heavy calcification of the LAD, and CTO of the LCX (). We first treated the LAD with standard stenting (). Computed tomography after successful revascularization of LAD revealed a short and mildly calcified CTO; and a stump was revealed after sending out the small branch (). Thereafter, we tried to treat the LCX-CTO. The middle LCX was occluded with a Rentrop grade 2 collateral flow from the posterolateral branch channel and the apical channel (, Videos –). However, interventional collateral channels were unclear.
We started PCI with the antegrade approach. We engaged an SPB 3.0, 8Fr (ASAHI Intecc, Nagoya, Japan) in the left coronary artery and progressed with the XT-R (ASAHI Intecc) supported by Corsair Pro (ASAHI Intecc) into the CTO stump. However, stiff wires and the parallel wire technique resulted in subintimal wiring (). We chose to convert to the retrograde approach. Tip injection revealed that the apical channel was connected to the posterolateral branch (). The SUOH 03 (ASAHI Intecc) passed the channel and bidirectional angiography revealed the short CTO length (), and the Gaia 2nd (ASAHI Intecc) directly crossed the CTO lesion (). Intravascular ultrasound imaging confirmed that the retrograde wire was in the true lumen (). However, a Mizuki (KANEKA MEDIX, Osaka, Japan) microcatheter could not pass the CTO lesion despite wire trapping by balloon catheter in the middle of the LCX. Even after the progression of the Gaia 2nd directly into the guiding catheter, the microcatheter could not pass the CTO lesion. Attempts were made to instead use the new Caravel microcatheter (ASAHI Intecc), which is thinner and has a softer body; however, it could not pass the CTO lesion as well. Thus, we planned to catch the retrograde wire with a snaring catheter, but that did not work. Therefore, we performed the rendezvous technique that meant full insertion of the retrograde Gaia 2nd into the antegrade Corsair Pro (, ). The antegrade Corsair Pro could advance into the CTO, tracking on the retrograde guidewire, but could not pass through the CTO completely (). Then, we tried the chasing wire technique, pushing the antegrade wire and pulling the retrograde wire simultaneously (, ). We chose the SION black (ASAHI Intecc) polymer jacket wire as an antegrade wire expecting smooth tracking along the route made by the retrograde guidewire before reocclusion. These techniques resulted in antegrade CTO crossing. We subsequently dilated the CTO with a small balloon and deployed the drug-eluting stent as usual (, Videos –).
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pmc-6232806-1
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A 76-year-old Caucasian female with a past medical history of chronic obstructive pulmonary disease (COPD), hypertension, and osteoarthritis had developed a COPD exacerbation requiring steroids and antibiotics. Her primary care physician ordered a chest X-ray (CXR) due to persistent cough and abnormal breath sounds on physical exam. The CXR revealed a moderate-sized infiltrate in the inferior portion of lingular segment which likely represented a pneumonic infiltrate. She was treated with a 10-day course of antibiotics. Repeat chest X-ray revealed minimal clearing of parenchymal infiltrate from the lingular segment. A subsequent computed tomography (CT) scan of the chest showed evidence of residual infiltrative changes involving the right middle lobe as well as the lingular division of the left upper lobe. There was also evidence of diffuse low-attenuation density involving the mediastinum highly suggestive of diffuse adenopathy which was concerning for lymphoma.
At initial consultation by oncology, her vital signs were stable, and she denied B symptoms including fevers, night sweats, and weight loss. She denied any hemoptysis or worsening shortness of breath. Physical exam was unremarkable with no palpable cervical, axillary, or inguinal adenopathy or hepatosplenomegaly, and respiratory exam was clear to auscultation bilaterally. CBC with differential showed a white blood cell count of 4.4 × 10 mm3 with an absolute lymphocyte count of 0.66 × 10 mm3, hemoglobin of 13.7 gm/dL, and platelet count of 178 × 10 mm3. She had an unremarkable complete metabolic panel (CMP) and mildly elevated lactate dehydrogenase (LDH) at 235. Due to concern for lymphoma and findings on CT of the chest, a PET/CT was ordered which showed an infiltrative mass in the mediastinum with diffuse uptake (maximum standardized uptake value (SUV) 5.94 ().
There were small lymph nodes in the left axilla showing low level uptake with maximum SUV 1.73 and 1.52, respectively. Finally, there was a hypermetabolic mass within the left iliac bone with a maximum SUV 11.71 ().
She underwent an endobronchial ultrasound and transbronchial biopsy of station 7 lymph node which revealed lymphoid tissue composed of small, mitotically inactive cells with round to slightly irregular nuclear contours and scant cytoplasm ().
Flow cytometric analysis demonstrated an abnormal CD5+ B cell population. Immunohistochemical stains showed that the cells were positive for CD20 () and CD5 () and negative for Cyclin D1 ().
Scattered CD3 positive T cells were also present. The morphology and phenotype supported the diagnosis of small lymphocytic leukemia (SLL). Since SLL does not typically present with bone lesions and there was concern for another primary cancer, a CT-guided biopsy was performed of the PET avid left iliac bone. Pathology from that biopsy showed both bone and marrow with involvement of CLL/SLL ().
Flow cytometry from the left iliac bone biopsy revealed monoclonal kappa light chain restricted B-cell population phenotypically consistent with CLL/SLL ().
No specific abnormalities were detected by CLL fluorescence in situ hybridization (FISH) including centromere 12, 13q14 (DLEU1), ATM/11q, TP53/17p13, and CCNDQ/IGH–t(11; 14).
Currently, she does not have cytopenias, B symptoms, or bulky disease; however, there was concern that the mediastinal adenopathy may be contributing to her pulmonary symptoms and that the left hip lesion was causing discomfort. Consequently, systemic therapy was offered as was radiation to the hip; however, the patient declined and opted for observation and close surveillance. She will return for further evaluation of symptoms and laboratory data in 2 months.
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pmc-6232809-1
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A 75-year-old woman with cough, rhinorrhea, sore throat, and appetite loss was evaluated at our hospital for anterior cervical pain and thyrotoxicosis. The patient's height was 145 cm, and her body weight was 40.0 kg. At her first visit to our hospital, her blood pressure was 137/81 mmHg, with a regular pulse rate of 116 beats/min. Her body temperature was 37.0°C. An electrocardiogram showed sinus tachycardia. She had no family history of autoimmune thyroid diseases. Nor did she take any medicines.
Her neck pain initially appeared on the left side and subsequently moved to the right side. Laboratory data showed a normal white cell count (8,290 cells /µL) and slightly elevated C-reactive protein (3.94 mg/dL) and alkaline phosphatase (460 U/L) levels (). Thyroid hormone levels were also elevated (free triiodothyronine, 20.27 pg/mL; free thyroxine, 6.53 ng/dL; thyroglobulin, 183 ng/mL), whereas thyroid-stimulating hormone (TSH) was undetectable. Ultrasonography (US) of the thyroid revealed heterogeneous and hypoechoic areas, which are features of subacute thyroiditis, in both thyroid lobes (). Biopsy was not performed.
The patient was treated with prednisolone (PSL, 20 mg/day), and her neck pain disappeared shortly thereafter. Two weeks after treatment initiation, we found strong anti-TSH receptor antibody (TRAb) and anti-thyroid-stimulating antibody positivity (). The PSL dose was gradually tapered, and thyroid hormone levels decreased, although they were still above normal 6 weeks after treatment start (). At this time, the patient received both PSL (10 mg/day) and methimazole (MMI, 10 mg/day). Four weeks later, thyroid hormone levels improved, and US showed hypervascularity and fewer hypoechoic areas in the thyroid. However liver function worsened, and MMI treatment was therefore stopped. One week after MMI withdrawal, the damaged liver had recovered, but the thyroid hormone levels were again elevated. Propylthiouracil (PTU, 100 mg/day) was administered, and levothyroxine (25 µg/day) was later added to control thyroid function.
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pmc-6232813-1
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The 64-year-old female patient was initially transferred to our hospital by a smaller district clinic for further treatment after a CT-scan revealed a large left-sided kidney-stone (2 cm) accompanied by an obstructive pyelonephritis. A Double-J-Stent was placed and after successful antibiotic therapy of the pyelonephritis the patient was released from the hospitalization in order to perform a ureterorenoscopic lithotripsy and stone-extraction in an outpatient setting 3 weeks later. Due to the stone's size a complete stone-extraction was not possible in one instance which led us to a second ureterorenoscopy 5 weeks after the initial consultation.
This second procedure started uneventfully with the cystoscopic removal of the Double-J-Stent and the insertion of a guide-wire. Following this the cystoscope was removed in order to insert a standard silicone Ch 12 Foley-Catheter (Nelaton-Tip) blocked with 2.5ml NaCl as it is custom for semirigid ureterorenoscopy in our clinic. Upon entering the bladder with the semirigid instrument, the position of the Foley-Catheter seemed suspicious. Further inspection revealed that the recently placed bladder-catheter led directly into the left-sided ureter. The balloon block was deflated immediately and the catheter was repositioned correctly into the bladder.
As demonstrated in Figures and , the following ureteroscopy and retrograde ureterography revealed a proximal partial rupture of the left ureter. We therefore decided to reinsert a Double-J-Stent and end the procedure. The already established antibiotic therapy with Ciprofloxacin due to the initial obstructive pyelonephritis preoperatively was continued for another week.
6 weeks later the patient was readmitted for control and in order to complete the stone-extraction. The initially ruptured ureter had recovered completely without contrast-leakage in the retrograde ureterography. The stone-extraction was thus finished without any further intraoperative complications. During the postoperative hospitalization, however, the patient developed a urinary infection which we considered to be caused by the vaporization of the colonized renal stones during the procedure but not directly associated with the initial ureteral injury.
During that hospitalization the Double-J-Stent which was exchanged during the last ureterorenoscopy was also removed. Blood-tests showed unsuspicious renal function and further sonographic controls proved normal renal drainage.
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pmc-6232817-1
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A 66-year-old Japanese woman was referred to the Department of Neurology for investigation of aphasia. The patient had no previous disease history and does not take any medications. Laboratory testing revealed decreased levels of vitamin B12 at 107 pg/mL (normal range: 257-989 pg/mL), for which she underwent esophagogastroduodenoscopy. Increased levels of rheumatoid factor at 38.9 IU/mL, hemoglobin A1c at 6.5%, and gastrin at 1,016 pg/mL were also noted. The number of red blood cells and the hemoglobin levels were within the normal ranges. She was positive for anti-intrinsic factor antibody and antiparietal cell antibody.
Esophagogastroduodenoscopy revealed gastric atrophy predominantly in the fornix () and in the body (), whereas atrophic changes were not evident in the antrum endoscopically (). Close-up observation of the gastric fornix showed multiple, slightly elevated, round, white substances (). Magnified observation with narrow-band imaging revealed microvasculature on its surface, suggesting deposition of the white substance within the mucosa (). Two biopsy samples were endoscopically taken from the fornix mucosa with the white substance. Three additional biopsies were done on the mucosa of the middle body, the lower body, and the antrum of the stomach, where the white substance was absent. Two biopsy specimens taken from the gastric mucosa that contained white substance revealed cystic dilatation of the gastric glands (). In contrast to this, there was no cystic dilatation in the glands of the gastric mucosa specimens where the white globe appearance was not observed. A prominent decrease in parietal cells was also noted. Parietal cell protrusion was absent. There were no Helicobacter pylori pathologically. We diagnosed autoimmune atrophic gastritis based on the serology, endoscopy, and pathology results.
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pmc-6232817-2
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An 81-year-old Japanese woman underwent esophagogastroduodenoscopy for annual screening purposes. The patient had been taking amlodipine for hypertension. Although she had undergone appendectomy at 30 years of age, she had no other history of abdominal diseases. Esophagogastroduodenoscopy showed diffuse gastric atrophy; the atrophic changes were more prominent in the fornix () than in the antrum (). After indigo carmine spraying, we noticed multiple, slightly elevated, round, white substances in the gastric fornix and body (Figures and ). Microvasculature was also noted on its surface as seen on the magnified observation with narrow-band imaging (). Biopsy from the gastric mucosa showed cystically dilated gastric glands (Figures –). Immunohistochemically, pepsinogen-positive cells were present (), whereas H+/K+ ATPase-positive cells were absent ().
Laboratory testing revealed normocytic anemia: hemoglobin was 10.2 g/dL, mean corpuscular volume was 92.5 fL, and mean corpuscular hemoglobin was 29.7 pg. Gastrin level was elevated at 392 pg/mL. Although the level of folic acid was decreased at 2.8 ng/mL, vitamin B12 level was within the normal range. The patient was positive for antiparietal cell antibody, while anti-intrinsic factor antibody was negative. She tested negative for urea breath test and H. pylori IgG antibody. Consequently, we diagnosed her as autoimmune atrophic gastritis.
The patient underwent esophagogastroduodenoscopy 12 months later. White substances were not detected in the gastric mucosa, even in the gastric fornix and body ().
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pmc-6232831-1
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A 21-year-old woman with no significant medical history except for treatment for right breast abscess two months prior, presented to the hospital with one week of fever, chills, myalgias, nausea, vomiting, diarrhea, cough, and progressive shortness of breath. The diarrhea was nonbloody and watery in consistency with a frequency of four episodes a day. She denied any chest pain, facial or leg swelling, weakness, headache, or dizziness. She stated her son had an unspecified illness recently that has resolved but denied any other sick contacts. She was found to be febrile with a temperature of 102.0°F, hypotensive with a blood pressure of 82/56 mmHg, and tachycardic at a rate of 149 bpm. A full physical exam was benign except for axillary and cervical lymphadenopathy. Laboratory workup revealed segmented neutrophil predominant leukocytosis, elevated levels of troponin (2.45 ng/ml), BNP (457.2 pg/ml), and d-dimer (6.72 µg/ml). Electrocardiogram (EKG) demonstrated sinus tachycardia with possible left atrial enlargement. Vasopressor support and unfractionated heparin drip were initiated, and the patient was admitted to the intensive care unit. Subsequent imaging showed a low-probability VQ scan for pulmonary embolism, and severe diffuse myocardial hypokinesis with left ventricular ejection fraction (LVEF) of 20–25% without pericardial effusion on 2D Echo ().
While CT abdomen demonstrated generalized lymphadenopathy and mild hepatosplenomegaly, chest X-ray was negative for any acute pathology on admission. Detailed rheumatologic workup was unremarkable, including antinuclear antibody (ANA), double-stranded DNA, antiproteinase 3, antimyeloperoxidase, and C3 and C4 levels. Right heart catheterization was performed and demonstrated elevated wedge pressure, pulmonary arterial pressure, and right ventricular pressure consistent with acute left ventricular failure and secondary pulmonary arterial hypertension. With a presumptive diagnosis of fulminant acute myocarditis, right ventricular endomyocardial biopsy (EMB) was performed disclosing a lymphocytic infiltrate with focal myocyte necrosis along with immunohistochemical stain for CD3 demonstrating presence of T cells ().
Serum Polymerase chain reaction (PCR) showed positivity for rhinovirus and Coxsackie viruses and further antibody titers confirmed significantly high but varying titer levels specific to six Coxsackie serotypes: B1 (1 : 32), B2 (1 :16), B3 (1 : 8), B4 (1 : 8), B5 (1 : 64), and B6 (1 : 64). A multidisciplinary team of cardiology, infectious disease, nephrology, and rheumatology initiated treatment with high dose steroid therapy and metoprolol. The hospital course was complicated by worsening acute kidney injury (AKI) and rhabdomyolysis with serum phosphocreatine kinase (CPK) levels as high as 21,867, both of which improved with IV fluid therapy. The patient's clinical condition progressively improved, with almost complete recovery of LVEF to 40–45%. The patient was downgraded to the medical floors and was stable for discharge by hospital day 8. The patient was discharged on lisinopril and metoprolol for compensated heart failure with a plan for close follow-up with cardiology, nephrology, and primary care. Repeat 2D-Echo done one month after discharge revealed complete resolution of systolic function with LVEF of 55–60%.
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pmc-6232949-1
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A 74-year-old man was evaluated by dermatology for a suspicious painless mass located over the posterior aspect of his right trapezius muscle. The mass had been present for at least 3 years but was noted to have had a rapid increase in size within the last 2 months prior to presentation. He denied any associated symptoms or recent changes in his health. Medical and surgical histories were significant for actinic keratoses, atrial fibrillation with cardiac ablation and placement of a permanent pacemaker, coronary artery disease, hypertension, hyperlipidemia, and benign prostatic hypertrophy. He denied any family history of malignancy, other than actinic keratoses. On examination, he was noted to have a palpable, nontender, mobile mass over the posterior aspect of his right upper trapezius muscle measuring approximately 3 × 3 cm. An incisional biopsy was performed by dermatology. Pathology revealed cellular spindle cell tumor without necrosis but with up to 6 mitoses/10 HPF. In addition, IHC staining was positive for CD34 and CD99. The histopathology was reviewed and the diagnosis of SFT was confirmed by the Department of Pathology by performing IHC staining for STAT6.
The patient was then seen in the surgical oncology office for further discussion and management. He was found to have a 3.5 × 1.2-cm mass with an overlying healing scar from his incisional biopsy. There was no evidence of satellitosis. Computed tomography of the chest, abdomen, and pelvis was performed to determine the extent of the tumor and revealed no evidence of metastatic disease. Prior to wide local excision, the patient was evaluated by the Multidisciplinary Cutaneous Oncology Clinic for any additional treatment recommendations. Neoadjuvant therapies were not recommended.
A full-thickness, wide local excision with 1-cm margins was performed. Additional trapezius muscle was taken for an oncological boundary of safety. All specimens were submitted to pathology. The primary resection defect measured 6.1 × 5.4 × 3.6 cm and was reconstructed with a local rotational-advancement flap.
Postoperatively, the flap reconstruction healed well without complication. Permanent pathology revealed positive deep margins with residual SFT. After extensive discussion with the patient, the decision was made to pursue adjuvant radiation therapy and forgo a secondary surgery. Radiation oncology plans for 30 treatments.
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pmc-6232950-1
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An 8-year-old boy presented with an extensive, large, black, hairy skin patch over the left periorbital area, forehead, cheek, and nose since birth. There was no family history of similar lesions or skin cancer. The patient had no neurological symptoms and was not taking any medications. Examination revealed a large pigmented patch, measuring approximately 13 cm in its greatest dimension on the left periorbital area and extended to cover nearly half of the face (). There was no increase in the size or change in color of the lesion since birth, and there was no pain, itching, or discharge. No other satellite lesions were present over the body, and there were no associated congenital anomalies. Parents’ counseling indicated that the lesion was affecting his school and social activities.
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pmc-6233252-1
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A 64-year-old woman visited our hospital with a 1-month history of a 1-cm dark red nodule in her right breast. Four years before, she underwent BCS and axillary lymph node dissection for right breast cancer followed by endocrine therapy and radiation therapy. The nodule was diagnosed as angiosarcoma by skin biopsy. A variety of image examination revealed a mass of 27 × 13 mm in outer lower lesion of her right breast, and the surrounding skin was markedly thickened (Fig. ). Mapping biopsy 2 cm from the edge of the nodule revealed tumor invasion in all five sites examined, while mapping biopsy at 5 cm or 10 cm revealed no tumor invasion in any of the six sites examined (Fig. a).
Total mastectomy with extensive skin resection (30 × 22 cm) was performed. The resection line was 10 cm from the edge of tumor. To repair a large skin defect, a wide skin graft using abdominal skin was performed. The pathological diagnosis was angiosarcoma, 45 × 40 × 20 mm in size (Fig. b, c). The surgical margins were completely free from tumor cells. Postoperative chemotherapy (weekly paclitaxel, 80 mg/m2 × 6 cycles) was administered, and the patient has experienced no recurrence for 6 years, 3 months.
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pmc-6233252-2
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A 67-year-old woman had undergone BCS and sentinel lymph node biopsy for left breast cancer followed by chemotherapy, anti-HER2 therapy, and radiation therapy 3 years before. She visited another hospital with a 3-month history of a dark red nodule in her left breast. The nodule had been diagnosed as angiosarcoma by open biopsy by a dermatologist (Fig. a). Immunohistochemistry such as CD31 and CD34 were positive. She then consulted our department for surgical treatment. We could not point out obvious abnormal findings in imaging findings.
Seven out of nine points of a mapping biopsy 5 cm from the surgical trace revealed atypical endothelial cells (Fig. b), while three out of ten points of mapping biopsy at 10 cm revealed atypical endothelial cells (Fig. c).
She underwent left mastectomy with extensive skin resection (25 × 20 cm). The resection range exceeded the three sites at which atypical endothelial cells were observed, and in other places, a range of 10 cm from the surgical trace was used. To repair a large skin defect, a wide skin graft from the thigh was performed.
Atypical endothelial cells were observed in resected specimens, but the degree of atypia was less than that of the primary tumor. Atypical endothelial cells were not observed in the resection margin (Fig. ).
Postoperative chemotherapy (nab-paclitaxel, 260 mg/m2 × 4 cycles) was administered, and the patient has experienced no recurrence for 5 years.
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pmc-6233277-1
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A 26-year-old man who underwent bilateral LASIK at 2009 developed bilateral post-LASIK keratectasia 2 years later. In April 2015, he came to our clinic for treatment of the left eye due to in tolerance to rigid gas permeable contact lenses (RGP). On examination, he had an uncorrected distance visual acuity (UDVA) of 20/200 in the right eye, which improved to 20/50 with a refractive correction of − 3.25 / -5.00 @ 160 degrees. His left eye had a UDVA of 20/200, which improved to 20/63 with a refractive correction of − 3.50 / -5.50 @ 100 degrees. Central corneal pachymetry was 395 μm in the right eye and 324 μm in the left eye. Corneal topography showed an inferior steepening (difference inferior-superior of around 13.40 D) in the right eye with a Sim K of 48.80 D at approximately 1.3 degrees and 55.80 D at approximately 91.3 degrees, and a diffuse corneal steepening more noticeable in the superior cornea (difference superior-inferior of around 4.80 D) with a Sim K of 57.50 D at approximately 5.3 degrees and 67.20 D at approximately 95.3 degrees in the left eye (Fig. a&b). As the patient’s corneal thickness was exceedingly thin at less than 400 μm, a CXL procedure was not recommended []. A lenticule addition procedure was approved by the Ethics Committee of the Fudan University EENT Hospital Review Board. After a written informed consent from the donor and recipient patients, the donor patient received blood testing for human immunodeficiency virus, hepatitis B and C viruses, blood glucose, rapid plasma reagin, and Treponema pallidum particle agglutination, and all results were normal. After that, the recipient patient underwent lenticule addition in his left eye. A maximum (central thickness) of 77 μm and minimum (peripheral thickness) of 10 μm lenticule was obtained the same day from a myopic SMILE of − 0.75 / -2.75 @ 180 degrees using the VisuMax femtosecond laser (Carl Zeiss Meditec, Jena, Germany). A sinskey hook was used to open the edge of the original flap, and a blunt spatula was used to lift the flap. The fresh lenticule was immediately added into the stroma, and carefully centered onto the apex of the cornea. Then the flap was repositioned and a bandage contact lens was applied.
Postoperative topical medication consisted of levofloxacin 4 times daily for 3 days, fluorometholone 0.1% 8 times daily, tapered to once daily over a period of 24 days, and a tear supplement 4 times daily for 1 month.
Four months after lenticule addition, the patient underwent CXL procedure of the left eye, as he had a sufficient corneal thickness of 412 μm. ParaCel (Avedro, Waltham, MA, USA) containing 0.25% riboflavin-5-phosphate, hydroxypropyl methylcellulose, sodium edetate, trometamol, benzalkonium chloride, and NaCl in a corneal epithelial trephine (Model 52503B; 66 Vision-Tech, Suzhou, China) was used to completely cover the cornea for a total of 4 min. The cornea was then rinsed completely with VibeX Xtra (Avedro) containing 0.25% riboflavin-5-phosphate and NaCl, and VibeX Xtra was used in the corneal epithelial trephine for a total of 6 min. After using the epithelial trephine, the cornea was rinsed completely with balanced salt solution (BSS). Ultraviolet treatment was conducted using the KXL System (Avedro). The treatment protocol consisted of pulsed illumination for 1 s using 45 mW/cm2 for a surface dose of 7.2 J, and frequency of the pulses was 50 Hz. The ultraviolet treatment procedure lasted for 5 min and 20 s. The cornea was then rinsed completely with BSS, and a bandage contact lens was applied. Antibiotic drops were administered for 1 week, and fluorometholone 0.1% were applied for 16 days (four times a day initially, then reduced once every 4 days). A tear supplement was also prescribed for 4 times per day for 1 month.
The patient had a stable UDVA of 20/125 and a best spectacle-corrected distance visual acuity (CDVA) of 20/40 with unchanging refraction of − 5.00 / -6.00 @ 100 degrees from 1 month after lenticule addition to 30 months postoperatively in his left eye. There were no visual abnormalities (such as halos or diplopia), corneal haze, or rejection observed under slit-lamp examination throughout the follow-up period (Fig. ).
Differential maps of Scheimpflug corneal topography at 30 months after surgery are shown in Fig. . The variation curve of front corneal K1, K2, and Kmax values are shown in Fig. a, which increased by 2.1 D, 4.5 D, and 7.8 D, respectively, at postoperative 30 months relative to preoperative values. Mean radius of the posterior curvature was 3.51 mm before surgery and 3.50 mm, 3.47 mm, 3.49 mm, 3.49 mm, and 3.55 at 1, 5, 9, 15, and 30 months post-surgery, respectively. The greatest posterior elevation was + 140 μm before surgery and + 170 μm, + 194 μm, + 154 μm, + 158 μm, and + 141 μm at 1, 5, 9, 15, 30 months post-surgery, respectively, showing an initial increase and then gradual decrease.
Time-dependent changes of corneal thickness measured with a Pentacam (Oculus Optikgeräte, Wetzlar, Germany) preoperatively and 1, 5, 9, 15, 30 months post-op are shown in Fig. b. Corneal thickness initially increased, then stabilized at 9 months after the lenticule addition, with a total increase of 73 μm at the pupil center.
The optical coherence topography (OCT) images (Fig. ) showed a clear lenticule at postoperative day 1. At 5 months follow-up, the lenticule demarcation lines became ambiguous and the density of the lenticule was similar to that of the surrounding corneal stroma.
In vivo confocal microscopy (IVCM) showed that both the anterior and posterior lenticule interfaces were detectable, characteristic of an absence or decrease in keratocytes and the presence of small particles of various brightness. Keratocytes in the implanted lenticule demonstrated elongated morphology and a decrease in number (Fig. ).
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pmc-6233285-1
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An 18 year old male fell from roof of a moving truck. The truck was moving at low speed and he fell on muddy floor from a height of approximately 12 feet and sustained impact over abdomen. He was admitted in Nepalgunj medical college where contrast enhanced computed tomography of abdomen showed grade III liver injury. He was managed conservatively with bed rest, 12 units of blood transfusion then discharged. He presented 15 days later to Alka Hospital emergency department with complaints of pain abdomen, excessive tiredness and vomiting of blood. His vitals in the emergency were pulse 100/min, blood pressure 90/60 mmHg, respiratory rate 20/min and temperature 99 degree F. Contrast enhanced computed tomography of abdomen was done which showed pseudoaneurysm of right hepatic artery with ruptured hematoma in segments VI, VII and VIII of liver (Figs. and ). Clot was present in the pelvis and lesser sac. Haemoglobin was 8 g/dL.
Patient was immediately transferred to intensive care unit and started on intravenous fluid, antibiotics. Blood transfusion was started but the patient became hemodynamically unstable. Patient was transferred to operation theatre and exploratory laparotomy was done. Intraoperative finding was approximately three litres of clot and blood in lesser sac and pelvis with approximately 5 cm laceration in segments VII and VIII of liver. Bleeding from the liver surface was controlled with gel foam packing, surgicell application. Drains were kept in Morrison’s pouch and pelvis. Five units of packed cell and two units of fresh frozen plasma was transfused intraoperatively. The pseudoaneurysm couldn’t be clipped intraoperatively due to the difficult location and hemodynamic instability of the patient. Postoperatively patient was transferred to intensive care unit. Patient was transfused with two units of blood postoperatively. Patient was transferred to the general ward on third postoperative day. Patient was discharged on eighth postoperative day.
On the 15th post operative day patient presented with hematemesis two episodes. On presentation to the out patient department, he was hemodynamically stable. CECT abdomen revealed hematoma in right lobe of liver with right hepatic artery pseudoaneurysm. Upper gastrointestinal endoscopy was done which confirmed hemobilia. Coil embolization of the pseudoaneurysm was planned and he was transferred to the interventional radiology unit in Tribhuvan University Teaching Hospital. Under aseptic precaution using femoral artery access coil embolization of hepatic artery pseudoaneurysm was done. Three coils were used for embolization (Figs. and ).
On the third post embolization day, patient developed high grade fever and persistent cough. Chest examination revealed basal crepitations on right side. Chest X ray and contrast enhanced computed tomography chest showed right lower lobe consolidation (Fig. ). Patient was started on intravenous antibiotics but the cough didn’t subside. Sputum content was sanguinous. On the seventh post embolisation day patient coughed up bile and a diagnosis of post traumatic bronchobiliary fistula was made. Bilioptysis increased with supine position. Endoscopic Retrograde Cholangiopancreatography was performed which showed an area of bile leak at right hepatic lobe close to the diaphragm at right anterior duct. Contrast was seen spilling into the right bronchial tree confirming fistulation. No obvious tract was noted. Modest sphincterotomy was performed and 10 Fr double pigtail stent was deployed. Post stenting the cough and bilioptysis resolved and the patient was discharged on the third day post endoscopic retrograde cholangiopancreatography. The stent was removed at 2 months. Follow up ultrasound scan at 2 months showed complete resolution of the hematoma.
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pmc-6233360-1
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A 71-year-old woman was referred to the Surgical Oncology Division at the Napoleão Laureano Hospital in João Pessoa, Brazil, presenting with a mass at the right side of the abdomen, associated with fever. She presented dyspeptic complaints and an isolated episode of acute pain in the upper abdomen, with radiation to the back, 20 days before the consultation. At admission, no signs of jaundice were found. An abdominal mass with elastic consistency was palpated at the right hypochondrium, 20 cm below the last rib. Laboratory tests indicated the following measures: hemoglobin 9.5 g/dL, carcinoembryonic antigen (CEA 1.96 ng/mL), serum C-reactive protein (123.8 mg/L), serum alkaline phosphatase (ALP 244 U/L), gamma-glutamyl transpeptidase (GGT 134 U/L), AST, ALT, albumin, and bilirubin within normal range. Abdominal ultrasonography (US) evidenced the presence of a solid, hypoechoic heterogeneous mass, measuring 14.2 × 9.5 × 13.8 cm, located on the right flank of the abdomen. Computed tomography (CT) showed extensive lobular formation (14.7 × 14.4 × 10.5 cm), exhibiting irregular enhancement and areas of fat density in the right side of the abdominal cavity, without cleavage plan with the gallbladder. The mass was adherent to the inferior border of the right hepatic lobe, bulging the ipsilateral abdominal wall and compressing the transverse colon, modifying its anatomic position (Fig. ). Three large gallbladder stones were identified (Fig. ). The CT report suggested gallbladder liposarcoma and cholelithiasis. Thoracic CT scan was normal. Based on these findings, on 3 February 2018, a laparotomy was indicated.
An extensive gallbladder tumor associated with focal liver invasion was observed (Fig. ). The cystic and biliary ducts were free from neoplastic invasion, and there were no signs of peritoneal spread nor of other organ metastases (Fig. ). A cholecystectomy associated with resection of segments IV-B and V of the liver was done (Fig. ). Intraoperative frozen sections of the opened excised tumor (Fig. ) were compatible with gallbladder sarcoma. Therefore, transoperative lymphadenectomy was not performed.
The patient was released 7 days after the surgical procedure and the anatomopathological examination and immunohistochemistry confirmed dedifferentiated liposarcoma (Fig. ) with foci of heterologous leiomyosarcomatous differentiation (Fig. ) and undifferentiated fusocellular areas of high histological grade (Fig. ), stage T3N0M0. Other immunohistochemical staining were studied, such as Pan-cytokeratin (AE1/AE3), epithelial membrane antigen (EMA), and high molecular weight cytokeratin (34betaE12), in order to refute the possibility of a carcinosarcoma of the gallbladder. All of them were negative.
At 8 months after the procedure, the patient is free of disease, presenting no signs of recurrence nor metastasis (Fig. ).
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pmc-6233374-1
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A 31-year-old white woman presented with severe burning pain with tingling sensation and asymmetric weakness of the lower limbs that, over a six-month period, gradually worsened and progressed to involve the upper limbs; she was then unable to walk or eat alone. She had been diagnosed with HIV infection five years earlier at another tertiary care hospital but refused follow-up.
On admission, she was undernourished and her neurological examination revealed: lethargy, disorientation and psychomotor slowing; asymmetrically diminished motor strength (Medical Research Council Scale) in the four limbs (grade 2/5 in right upper limb extension; grade 3/5 in bilateral lower limb extension; grade 4/5 in the remaining); symmetrical deep tendon reflexes apart from absent right brachioradialis and bilateral patellar reflexes; impaired pin-prick sensibility in the right ulnar and radial distribution. The remainder physical examination was unremarkable.
Brain MRI (Fig. ) was consistent with HIV encephalopathy and electromyography (EMG) with the diagnosis of MNM (severe confluent multifocal demyelination and axonal loss in both upper and lower limbs).
CD4 cell count was 75 cells/μL (8%) and HIV RNA was 633000 copies/mL. CMV DNA in blood was 64000 copies/mL; CMV antigen was negative. CMV IgG antibodies were positive and IgM antibodies negative; furthermore, electronic medical records from five years earlier confirmed prior CMV IgG seropositivity, suggesting CMV reactivation. Cerebrospinal fluid (CSF) analysis revealed 7 cells, protein of 1.19 g/dL and glucose of 49 mg/dL; negative bacterial and fungal cultures; positive CMV DNA (14400 cp/mL) and HIV RNA (184222 cp/mL). Gastrointestinal involvement (esophageal and colonic) by disseminated CMV disease was histologically documented, even though the patient reported no related symptoms; retinal involvement was excluded.
With disseminated CMV disease as the most likely cause of MNM, she started IV ganciclovir (5 mg/kg every 12 h). Due to no significant clinical improvement, treatment was intensified 7 days later with the association of IV foscarnet (90 mg/kg every 12 h). Combination therapy was maintained for three weeks, after which CMV DNA became undetectable in blood (she refused a new lumbar puncture) and oral valganciclovir 900 mg/day was started as maintenance therapy. She initiated ART with emtricitabine/tenofovir and dolutegravir following two weeks of combination therapy targeted at CMV disease and slow, but obvious, clinical response. Her neurological symptoms, both neurocognitive and motor, gradually improved and she began a rehabilitation program. She was transferred to a rehabilitation center and discharged after two months.
Follow up at three months of ART initiation showed CD4 cell count improvement (313 cells/μL (27%)) and undetectable HIV RNA. After 6 months of sustained immune recovery (CD4 cell count 722 cells/μL (24%)) and virologic suppression, maintenance therapy with valganciclovir was stopped.
At 12 months of initial symptoms, she showed no neurocognitive impairment and was able to return to her normal daily activities. Motor strength improved globally to normality besides grade 4/5 in right-hand fingers extension and in the lower limb extension; pin-prick sensibility remained impaired in the right upper limb. She reported neuropathic pain in the lower limbs, which was managed with pregabalin 225 mg twice daily. EMG was consistent with nerve regeneration in the upper limbs and, to a lesser extent, in the right lower limb.
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pmc-6233383-1
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A 53-year-old Chinese woman was admitted for a 6-month history of foamy urine. Two months before admission, her urinalysis revealed proteinuria 2+ without hematuria. Protein excretion was 2.76 to 3.15 g/24 h. Her serum albumin was 40.1 g/L (normal range: 40–55 g/L), and serum creatinine was 2.20 to 2.50 mg/dl (normal range: 0.50–1.50 mg/dl). Her serum immunoglobulin (Ig) G was 17.2 g/L (normal range: 7.23–16.85 g/L), IgA was 0.59 g/L (normal range: 0.69–3.82 g/L), and IgM was 0.83 g/L (normal range: 0.63–2.77 g/L). Monoclonal IgGκ spike was identified in the serum by immunofixation electrophoresis, and monoclonal IgGκ plus free κ light chain was identified in the urine. Bone marrow aspiration smear revealed 1% plasma cells. CD38, CD138 and CD56 positive cells accounted for 1.13% of bone marrow cells with κ light chain restricted expression as determined by bone marrow flow cytometry. The patient was then referred to our hospital for further evaluation.
She had a 4-year history of hypertension for which she was taking irbesartan. Family history was negative. On admission, the physical examination revealed a blood pressure of 113/65 mmHg, temperature of 36.5 °C, heart rate of 78/min, and respiratory rate of 18/min. No organomegaly was noticed. Other signs were normal.
After admission, urinalysis revealed proteinuria 1.27 g/24 h. The albumin creatinine ratio (ACR) was 751.40 mg/gCr (normal range: < 30 mg/gCr). The urine sediment examination was normal. The urine pH was 5.0, and the specific gravity was 1.007. The urine N-acetyl-β-D-glucosidase (NAG) was 12 U/L (normal range: 0–21 U/L), and α1-microglobulin was 86.1 mg/L (normal range: 0–12 mg/L). Urine glucose was negative. Other laboratory data revealed serum creatinine of 2.34 mg/dl, estimated glomerular filtration rate (eGFR) of 23.00 ml/min/1.73m2, serum total protein of 79.5 g/L (normal range: 65–85 g/L), and serum albumin of 42.6 g/L. The sizes of both kidneys were normal. Her serum calcium was 2.39 mmol/L (normal range: 2.11–2.52 mmol/L), phosphate was 1.22 mmol/L (normal range: 0.85–1.51 mmol/L) and the uric acid was 312 μmol/L (normal range: 90–360 μmol/L). Serum liver enzymes were normal. Her white blood cell count was 7.7 × 109 cells/L (normal range: 3.5–9.5 × 109 cells/L), hemoglobin was 148 g/L (normal range: 115–150 g/L) and the platelet count was 205 × 109 cells/L (normal range: 125–300 × 109 cells/L). The prothrombin time was 10.5 s (normal range: 9.0–11.5 s), the activated partial thromboplastin time was 28.9 s (normal range: 26.9–37.6 s) and the plasma fibrinogen level was 3.80 g/L (normal range: 2–4 g/L). She had type 1 cryoglobulinemia with IgGκ. Serum free κ chain was 35.4 mg/L (normal range: 3.30–19.40 mg/L), free λ chain was 16.8 mg/L (normal range: 5.71–26.3 mg/L), and the κ/λ ratio was 2.11 (normal range: 0.26–1.65). Cranial and pelvic bone X-rays did not indicate obvious bone destruction. Echocardiography and abdominal ultrasound were normal. Hepatitis B surface antigen (HBsAg), anti- hepatitis C virus (HCV), anti- human immunodeficiency virus (HIV) and Treponema pallidum antibody (TP-Ab) were all negative. Plasma complement 3 (C3) was 1.240 g/L (normal range: 0.60–1.50 g/L), and complement 4 (C4) was 0.268 g/L (normal range: 0.12–0.36 g/L). Anti-nuclear antibodies, anti-neutrophil cytoplasmic antibodies and anti- phospholipase A2 receptor (PLA2R) antibodies were all negative.
MGRS was suspected, but other glomerular diseases accompanied by monoclonal gammopathy of undetermined significance (MGUS) could not be excluded and can only be confirmed by renal biopsy. The patient underwent renal biopsy. Direct immunofluorescence (IF) examination of frozen renal tissue revealed no significant immune deposits and light chains(κ, λ) in the glomeruli, tubules and interstitium. Light microscopic examination showed that 12/29 glomeruli were globally sclerosed and 5/29 glomeruli showed segmental sclerosis with cytoplasmic vacuolization of podocytes (Fig. ). Other glomeruli were nearly normal. Tubular epithelial cells exhibited focal vacuolization and eosinophilic granules in the cytoplasm and focal loss of brush border with epithelial simplification (Fig. ). Tubular atrophy and interstitial fibrosis were minimal. There was mild interstitial infiltration of lymphocytes, monocytes and a few eosinophils. Mild arteriolar sclerosis and intimal fibrosis of the artery were observed. Congo red staining for amyloid was negative. Electron microscopic examination revealed rod- or rhomboid-shaped crystals in the podocytes (Fig. ) and proximal tubular epithelial cells (Fig. ). The histiocytes did not contain any crystal inclusions. Majority of the podocyte foot processes were effaced. No electron-dense deposits were observed in the glomeruli. Immuno-electron microscopy revealed κ light chain deposition in the crystals without λ light chain (Fig. ).
The patient was diagnosed with crystal-storing renal disease involving the podocytes and proximal tubular epithelial cells. She was transferred to the hematological department, and received 4 cycles of CBD (Bortezomib, dexamethasone and cyclophosphamide) protocol chemotherapy. Serum immunofixation electrophoresis still showed IgGκ and urine with IgGκ plus free κ light chain. The patient was considered to be resistant to CBD reatment and switched to Rd. (Lenalidomide and dexamethasone). The patient showed good compliance, and the treatment was well tolerated without clinically significant side effects. The patient was followed up for 12 months until now, and the serum creatinine was approximately 2.26 mg/dl with a proteinuria of 0.3–0.5 g/24 h.
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pmc-6233537-1
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A 71-year-old Caucasian man presented to our emergency room with vomiting and abdominal pain. He had been experiencing constipation and abdominal discomfort for a few weeks and had heard borborygmi in his intestine. He had noticed an occasional black stool during defecation. Due to persistent normocytic anemia, with hemoglobin levels below 100 g/L, he had had a gastroscopy, which revealed no abnormal conditions. He was also scheduled for a colonoscopy.
He had a history of arterial hypertension. Seven years earlier, he had malignant melanoma excised from his abdominal skin. Left-sided axillary lymphadenectomy was performed later due to positive sentinel node. For 6 years his clinical condition was stable. Then, a metastasis on his vocal cord and in his sternum was found, along with a suspicious lesion in his left breast. He underwent total laryngectomy and started receiving immunotherapy with vemurafenib and cobimetinib. Due to adverse side effects, including vomiting, weight loss, and phototoxicity, his therapy was adjusted to reduced dosages, which he was still receiving at the time of our encounter. A head, neck, and chest computed tomography (CT) scan performed for follow-up in another institution 1 month before admission to our department, demonstrated a stable disease.
In our emergency room he was stable. His abdomen was distended and diffusely tender on palpation, but without any signs of peritoneal irritation. An absence of bowel sounds was discovered on auscultation. An emergency CT scan was performed, demonstrating a 10 cm long segment of small bowel intussusception (Fig. ). The leading cause of intussusception was unclear, but the possibility of a Meckel’s diverticulum or a metastatic lesion was discussed.
After conservative measures and a nasogastric tube and intravenously administered fluids, he was taken to the operative theatre where an explorative laparotomy was done. His proximal small bowel was immensely distended, yet bowel motility was preserved and blood perfusion was good. Approximately 100 cm distally from the ligament of Treitz a jejuno-jejunal intussusception was found to be causing obstruction (Fig. ). At that point an intraluminal tumor was palpable. No other abnormal conditions or suspicious lesions were found in his abdomen. First, intussusception was manually resolved. A small enterotomy at the level of the tumor revealed a pedunculated formation, measuring 5 cm in diameter (Fig. ). Excision of the tumor along with the adherent mucosa was performed. The enterotomy was eventually closed with interrupted reabsorbable sutures. After the procedure he was admitted to our intensive care unit and a few days later to a normal hospital ward. Further hospital stay was uneventful and 13 days after admission he was discharged.
Histology of the tumor confirmed it to be a metastasis of malignant melanoma: S100, MelanA, and human melanoma black-45 (HMB-45), all positive. R0 resection was achieved. Further follow-up visits were scheduled with our patient’s treating oncologist at another institution. At the last visit, his clinical condition was stable and he resumed immunotherapy.
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pmc-6233546-1
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An 11-year-old girl was admitted to our hospital due to abdominal pain and diarrhea of 1 week’s duration. She had no medical history of abdominal trauma or surgery. In addition, she had no travel history and there were no pets at her home. Initial assessment of vital signs showed a blood pressure of 116/70 mmHg, a heart rate of 86 beats per minute, body temperature of 36 °C, respiratory rate of 20 breaths/min, and oxygen saturation of 99%, all of which were within normal range for her age. On physical examination, she had tenderness in the right lower quadrant of the abdomen without rebound tenderness. The spleen and liver were not palpable. Laboratory examinations yielded normal results with a leukocyte count of 6,050 cells/μL, hemoglobin concentration of 13.7 g/dL, platelet count of 318,000 platelets/μL, prothrombin time of 12.3 s, and activated partial thromboplastin time of 30.6 s. Initially, she was diagnosed with acute gastroenteritis, and a contrast-enhanced computed tomography (CT) scan of the abdomen was performed to rule out acute appendicitis. A CT scan of the abdomen revealed an enhancing mass, 61 × 54 × 65 mm in size and several subcentimeter enhancing nodules in the spleen, suggesting possible hemangioma, as well as diffuse edematous wall thickening in the colon (Fig. ). She was diagnosed with acute colitis and a giant splenic hemangioma that was found incidentally, and treated with intravenous hydration and medication for acute colitis. After the symptoms of acute colitis resolved, she received vaccinations for encapsulated bacteria including Haemophilus influenzae type b, Streptococcus pneumoniae, and Neisseria meningitidis.
Two weeks after completion of the vaccinations, the patient underwent splenic embolization at the interventional center by a clinically experienced interventional radiologist. The procedure was performed with the patient under general anesthesia and the electrocardiogram, blood pressure, and oxygen saturation with pulse oximetry were continuously monitored during the procedure.
The right common femoral artery was accessed under sonographic guide via the Seldinger technique and a 5-F arterial sheath was placed. A 5-Fr angiographic catheter (Yashiro Glidecath; Terumo, Tokyo, Japan) was used for access and to perform angiography of the celiac trunk and splenic artery. On celiac angiography, a giant hemangioma and multiple daughter nodules were identified in the spleen. Before performing the angiography, we had initially planned selectively embolize of the main mass and the large daughter nodules (Fig. a). However, numerous daughter nodules throughout the entire spleen were observed on angiography, and complete splenic artery embolization was performed. For splenic artery embolization, a 1.9-Fr microcatheter (Tellus; Asahi Intecc; Aichi, Japan) was inserted through the angiographic catheter and advanced through the distal splenic artery at the level of the hilum. Polyvinyl alcohol (Contour SE; Boston Scientific, Fremont, CA, USA) particles were initially used for splenic artery embolization and N-butyl cyanoacrylate (Histoacryl; Braun, Sempach, Switzerland) was additionally used for more complete embolization. We occluded the splenic artery at the distal level. Following embolization, angiography demonstrated complete occlusion of the splenic artery (Fig. b). There were no acute complications after splenic embolization including bleeding.
At 4 h post-embolization, she developed mild abdominal pain, which was managed with alternating acetaminophen and ketorolac. At 12 h post-embolization, she developed intermittent fever below 39 °C, which was managed with acetaminophen. Blood and urine cultures were subsequently performed. On day 5 post-splenic embolization, hematologic studies showed thrombocytosis, with a platelet count of 502,000/μL. On day 6, a contrast-enhanced CT scan of the abdomen revealed total infarction of the spleen. There were no complications observed, including splenic abscess or bleeding (Fig. ). On day 7, culture studies showed an absence of bacteria. The abdominal pain and fever had subsided, and the patient was discharged. During outpatient follow-up, the platelet counts peaked at 950,000/μL on day 20 post-splenic embolization and returned to normal 2 months after splenic embolization. No other complications related to the embolization, including pulmonary complications, severe infection, or portal vein thrombosis, occurred during 6 months of follow-up. The patient was prescribed daily prophylaxis with oral amoxicillin for 1 year post-embolization due to her functional asplenia.
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pmc-6233559-1
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A 73-year-old white male patient was referred to the hematology clinic due to a significantly elevated white blood cell (WBC) count that was detected following presentation with fatigue and drenching night sweats lasting 2 weeks. Night sweats and fatigue can be signs of an infection, malignancy, or hormonal abnormality, or they can be side effects of medication. For patients presenting with these symptoms, likely potential diagnoses include tuberculosis, HIV, abscesses, infective endocarditis, lymphoma or leukemia, hyperthyroidism, pheochromocytoma, or carcinoid syndrome.
The patient’s medical, surgical, social, and family histories are reported in Table . There were no relevant past interventions. To further evaluate and diagnose the patient’s condition, we performed a complete blood count (CBC; Table ) and peripheral blood smear. The peripheral blood smear showed a number of teardrop cells. Following the CBC and peripheral blood smear results, an abdominal ultrasound was performed and showed splenomegaly of approximately 16 cm. The lactate dehydrogenase level was also examined and found to be elevated at 1005 U/L.
The patient’s clinical presentation, elevated WBC count, splenomegaly, and peripheral blood smear results were suggestive of a myeloproliferative disorder, with CML suggested based on the peripheral blood smear and cytological analyses. To confirm a diagnosis of CML, a bone marrow biopsy and PCR test on peripheral blood for the BCR-ABL1 fusion gene were conducted. Examination of cells from the bone marrow biopsy showed hypercellular marrow, with increased megakaryocytes, increased and left-shifted granulopoiesis, markedly decreased erythropoiesis, eosinophilia, decreased iron, severe reticulin fibrosis, and approximately 5% blasts. A CD34 immunohistochemical stain showed scattered CD34-positive blasts comprising approximately 5% of the overall marrow cellularity, with variable distribution of blasts without clusters. A cytogenetic analysis could not be performed owing to a culture failure, likely resulting from a clotted specimen. However, a PCR test was positive for the BCR-ABL1 fusion gene.
The patient was in chronic phase of CML and according to his Sokal risk score, was classified as low risk. The Kaplan-Meier-estimated 5-year overall survival rate for patients in his age group (65–74 years old) diagnosed with CML in 2000 (before the introduction of TKIs) compared with those diagnosed with CML in 2005 (after the introduction of TKIs) was reported as 38.1% versus 51.2%, respectively (hazard ratio for mortality, 0.692; 95% CI, 0.518–0.924; P = .0126) []. Available treatments and their side effect profiles were discussed with the patient, and he elected to proceed with dasatinib treatment.
The patient was started on dasatinib 100 mg once daily. Treatment adherence and tolerability were reviewed during each of his follow-up visits to the clinic; the number of pills remaining, if any, was always verified with the patient. He tolerated the treatment well and within 2 months experienced a complete hematologic response. The patient’s response was monitored by evaluating BCR-ABL1 transcript levels; isolated RNA was reverse transcribed, after which the complementary DNA was amplified by RQ-PCR for the major and minor BCR-ABL1 fusion genes. The patient had no evidence of disease progression and achieved a molecular response of BCR-ABL1 < 10% on the IS during month 5 of treatment. For patients with this level of response, the NCCN recommends continuing the current treatment, with ongoing monitoring of response levels []. By approximately month 8 of treatment, BCR-ABL1 levels increased slightly from 2.40 to 3.59% on the IS; however, a subsequent assessment 4 weeks later showed a reduction of BCR-ABL1 levels to 2.99% on the IS.
Increasing BCR-ABL1 levels can be an early sign of treatment resistance []. In prior studies, a ≥ 2-fold increase in BCR-ABL1 levels in single or serial samples was shown to be predictive of BCR-ABL1 mutations [, ], which are a frequent cause of TKI resistance [, –]. The NCCN recommends additional testing in patients with a 1-log increase in BCR-ABL1 levels and loss of MMR to determine if a change in treatment is needed []. However, in this case, the patient’s increasing BCR-ABL1 levels at month 8 of treatment were below the 2-fold and 1-log thresholds, and they spontaneously improved by the subsequent assessment. At month 12 of treatment, a bone marrow biopsy revealed no increase in blasts (< 1%) and adequate erythropoiesis and granulopoiesis, while RQ-PCR showed a BCR-ABL1 level of 0.22% on the IS, which is close to a major molecular response (BCR-ABL1 ≤ 0.1% on the IS). The favorable results of the bone marrow biopsy and the RQ-PCR results indicated that the patient was responding well to treatment. The patient continued treatment with dasatinib (Fig. ).
At month 20 of dasatinib therapy, another increase in BCR-ABL1 levels was detected (from 0.32% on the IS at month 16 to 6.09% at month 20). However, the patient showed no clinical evidence of disease progression, remained on treatment with good adherence, and had normal CBC levels. He was therefore kept on dasatinib treatment, and his BCR-ABL1 levels were assessed again at month 21. This assessment showed that his BCR-ABL1 levels had increased further, to 12.77% on the IS. A bone marrow biopsy revealed no evidence of acute leukemia. Cytogenetic analysis showed that 10 of 20 cells were positive for the Philadelphia chromosome; 10 normal cells were observed. Unlike the earlier increase in BCR-ABL1 levels, this increase was substantial enough to trigger BCR-ABL1 mutational analysis despite the absence of clinical evidence of disease progression. Genetic sequencing of a bone marrow aspirate sample detected a V299L mutation in the BCR-ABL1 kinase domain. Low levels of an insertion event, during which 35 nucleotides from ABL1 intron 8 were inserted at the normal exon 8 to exon 9 splice junction, were also detected; the clinical significance of this is unknown. The NCCN recommends switching patients with V299L mutations to nilotinib []. In accordance with these treatment guidelines, the patient was switched to nilotinib 400 mg twice daily.
After starting nilotinib 400 mg twice daily, the patient developed abdominal pain, slightly elevated amylase and lipase levels, and profound fatigue. Due to these adverse events, the nilotinib dose was temporarily reduced to 200 mg twice daily and then escalated to a 300-mg twice-daily maintenance dose. RQ-PCR testing at month 18 revealed a BCR-ABL1 level of 0.00% on the IS, a greater reduction than was previously achieved with dasatinib. To date, the patient has remained on nilotinib 300 mg twice daily and has demonstrated good tolerability of the drug, no recurrence of abdominal pain or fatigue, and no clinical evidence of disease progression. BCR-ABL1 levels rose to 0.20% on the IS at month 21 of nilotinib but returned to 0.00% on the IS the following month. In the latest assessment, at month 34 of treatment, the patient had BCR-ABL1 levels of 0.30% on the IS, up from 0.00% on the IS at month 28. He showed no evidence of cytogenetic or hematologic relapse and is being periodically followed at the clinic per the NCCN guidelines [].
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pmc-6233589-1
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A 53-year-old female presented with intermittent abdominal distension and was admitted to the hospital. The patient had a history of chronic hepatitis B infection, and as a result, received antiviral therapy. No scleral icterus or xanthochromia was detected, Murphy’s sign was negative, and the patient’s performance status score was 1. Abnormal prothrombin, carcinoembryonic antigen (CEA) and alpha-fetoprotein (AFP) levels were within the normal range; however, cancer antigen 19–9 (CA19–9) level was increased to 66.81 U/ml, respectively. Magnetic resonance imaging (MRI) revealed a space-occupying lesion in the left liver, while no abnormal space-occupying lesions were found in the lungs, breast, gastrointestinal tract, or other areas that are prone to liver metastasis. Therefore, this lesion was considered a primary liver tumour. The patient underwent a curative resection in August 2016. The tumour tissues were sent for pathological evaluation, which indicated poorly differentiated adenocarcinoma. The tumour was 8*5.5*9.5 cm in size and was also necrotic and nodular with vessel invasion (Fig. and ); however, invasion of the nervous system or surgical margins was not observed. An immunohistochemical analysis revealed the following: AFP(−), CA-125(−), CD10(−), CD34(blood vessel+), CKpan(+), CK7(−), CK19(+), CK20(−), HCV(−), HBcAg(−), HBsAg(liver+) Ki-67(50%+), P53(90%+), TTF-1(−), vimentin(+), WT1(−), and Gly3(−). These results suggested a diagnosis of stage IIIB iCCA (pT2N1M0).
Two months after surgery, the patient underwent a computed tomography (CT) examination, which demonstrated a metastatic focus in the coelom (Fig. and ). The patient received chemotherapy consisting of gemcitabine and cisplatin (GP). After four cycles, positron emission tomography-computed tomography (PET-CT) showed an increase in the number of metastatic lesions in the coelom, which suggested disease progression (Fig. and ). With the patient’s consent, the tissue sample obtained during surgery was submitted for genomic alteration testing using a 450-gene panel as part of next generation sequencing (NGS); the tissue was also tested for the expression of PD-L1. Genomic testing showed the TP53 R249S, ATRX K1177, and RB1 K80 mutations, but no mutations were detected in the ERBB2 or KRAS genes. The tumour mutational burden was 19.3 mut/Mb and was defined as TMB-high. The MSI status was stable. Immunohistochemistry (IHC) results showed that the PD-L1 TPS of the tissue sample was 80%, which indicated high expression of PD-L1 (Fig. ). Moreover, MMR proteins were positive. With the patient’s consent, a combination therapy consisting of immunotherapy and chemotherapy was administered based on these findings. After treatment with pembrolizumab (150 mg q3w), oxaliplatin and tegafur [SOX] (oxaliplatin 130 mg/m2, d1, tegafur 60 mg BID, d1–14, q3w) for 4 cycles, CT showed that the lesions in the coelom had significantly decreased in size (Fig. and ). Subsequently, the patient received combination therapy with pembrolizumab and SOX for another 3 cycles. During the 5th cycle of combined therapy, the patient experienced third-degree neutropenia with fever. Considering the side effects of the chemotherapy, the situation was improved after G-CSF treatment was administered. Then, the dose of chemotherapy was lowered to 80%. The lesions in the coelom had nearly resolved on the CT scans in July 2017 (Fig. and ), which suggested that the disease was in complete remission. The patient received a total of 8 cycles of pembrolizumab+SOX and then received pembrolizumab maintenance monotherapy for 6 months. The drug treatment was stopped in February 2018 due to personal reasons. From July 2017 to May 2018, when was the patient was last followed-up, the cancer was in complete remission (CR) (Fig. and ), and the toxicity associated with immunotherapy was not obvious.
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pmc-6233954-1
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A 21-year-old male with a previous medical history of depression and no other medical comorbidities presented to the emergency department (ED) with a decreased level of consciousness after taking an amitriptyline overdose as a suicidal attempt. The patient was found to have a Glasgow Coma Scale (GCS) of three and was subsequently intubated and admitted to the intensive care unit (ICU).
Initial laboratory workup showed lactic acidosis, negative troponin, and normal kidney and liver functions. An arterial blood gas (ABG) was done, and the patient was found to have metabolic acidosis (pH 7.2) with respiratory compensation. The EKG showed a wide complex tachycardia with a ventricular rate of 146 bpm, a QRS complex duration of 118 msec, and a prolonged QTc at 576 with nonspecific ST-T wave changes. The initial transthoracic echo (TTE) revealed a preserved ejection fraction (EF) at 65% and no wall segment motion abnormalities. The patient was started on intravenous fluids and intravenous sodium bicarbonate with a target pH of 7.5-7.55. On day two of admission, our patient improved clinically and was taken off mechanical ventilation. The QRS complex and QTc began to shorten. However, cardiac troponin I levels started to rise with a peak of 4.08 µg/L. The patient developed a fever with a maximum body temperature of 312.1 K, an elevation in WBC count at 13.2 x 109/L (with an absence of peripheral eosinophilia), and an elevation in brain natriuretic peptide at 399 pg/ml. Erythrocyte sedimentation rate and C-reactive protein were also elevated at 46 mm/hr and 18 mg/L, respectively. Reviewing the history further, the patient reported the ingestion of 41 amitriptyline 50 mg tablets. He denied having any recent flu-like symptoms, no exposure to sick contacts, and a viral panel was negative for common viruses, including coxsackie and adenovirus. His only prescribed medication was amitriptyline and he did not use over-the-counter medications regularly. Amitriptyline levels were not obtained as the patient was admitted while fully conscious after ingesting 41 tablets; this was confirmed through a tablet count of his prescription bottle.
Cardiology service was consulted. Repeat TTE showed a mildly reduced EF at 45%-50%, mild to moderate pericardial effusion, and no wall segment motion abnormalities (Figure ).
Cardiac magnetic resonance (CMR) was done for a suspicion of acute myocarditis and revealed a moderately dilated left ventricle with mildly reduced EF at 45%, subtle enhancement of the basal inferolateral epicardium on delayed enhancement images (Figure ), non-territorial scattered areas of edema within the myocardium (Figure ), and moderate pericardial effusion. Findings were compatible with acute myocarditis. CMR was negative for coronary artery stenosis or an anomalous coronary artery origin as possible causes of ischemia or the elevated troponin level.
The patient was diagnosed with amitriptyline-induced cardiotoxicity in the form of drug-induced myocarditis with pericardial involvement. Supportive therapy with intravenous fluids, sodium bicarbonate, and the correction of electrolytes contributed to the clinical improvement. The patient recovered well and was discharged home after seven days of hospitalization. On the one month follow-up, the troponin level was repeated and was within normal limits. Repeat TTE demonstrated a normal left ventricular function with an EF of 65% and resolved pericardial effusion (Figure ).
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pmc-6234204-1
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In 1995, a 25-year-old lady was on her way to work riding a bicycle when she collided with a van parked on the bicycle lane. In the hospital, X-ray of cervical spine revealed C-6/C-7 dislocation. Clinical examination revealed C-8 AIS A tetraplegia. She was treated nonoperatively. Following rehabilitation, this patient had been managing her bladder by intermittent catheterisations performed by caregivers and intra-vesical instillation of oxybutynin 5 mg solution four times a day.
In 2014, this patient developed recurring bladder spasms and urine leakages in between intermittent catheterisations. She could not retain oxybutynin intra-vesical instillations; the solution would come out as soon as it went inside the bladder. This patient experienced symptoms of autonomic dysreflexia including blotches on her legs, sharp pain in her head, hot feeling on her face and bladder pain with bladder spasms. Once caregivers performed catheterisation, blotches went away and headache was relieved; feeling of warm sensation disappeared, but bladder pain persisted. She was prescribed mirabegron 50 mg once a day from June 2014 to January 2015; the patient felt that mirabegron did not work as it made no difference to urine leakages. She was then prescribed oxybutynin by mouth and transdermal application of oxybutynin from January 2015. This patient had been taking oral baclofen since 1995; baclofen had no effect on bladder spasms although oral baclofen reduced spasms in her back and legs.
This patient found it necessary to catheterise every couple of hours. Despite such frequent catheterisations, she had urinary leakages between catheterisation, which affected her quality of life. She found it difficult to cope physically and mentally with this situation. The patient did not want to live with a permanent catheter and urine bag, she had used a permanent catheter for social occasions but sometimes she would bypass. This patient had come to terms that she would be unable to walk but she could not accept to live with permanent urinary catheter and leg bag.
Videourodynamics was performed in October 2014; initial residual urine was small. The filling phase showed gross detrusor over-activity. The reflex volume was less than 70 ml; pdet went up to 50 cm H2O with spontaneous emptying. While doing stress leakage, there was no stress leakage; the bladder neck was well supported. The second fill showed exactly the same findings in spite of a very slow fill.
The patient’s first referral to the Urology Clinic focused on:Eligibility to have the bladder wall botulinum toxin injection at the tertiary care hospital. Confirmation of the problems the patient was experiencing and that relevant urology tests and video urodynamic studies had been performed and were current. Potential risk of getting autonomic dysreflexia and what measures needed to be in place before any procedure was performed. The patient was told she met the criteria for the Bladder botulinum toxin injection and would be put on the waiting list. Discussion about the procedure of botulinum toxin injection to the bladder, and how it is performed under local anaesthesia using flexible cystoscopy.
There was no conversation about the dose of Abobotulinum toxin A the patient should receive. The patient received no verbal communication or written literature regarding possibility of generalised muscle weakness occurring after bladder wall injection of Abobotulinum toxin A and potential impact of muscle weakness on her care needs. Risks outlined to the patient at the first procedure were: possibility of urine infection, passing blood in urine and slight spotting. The risk of generalised muscle weakness after Abobotulinum toxin A injections was not explained to the patient at any time before or after the procedure.
Abobotulinum Toxin A 1000 units were injected into the urinary bladder under local anaesthesia in May 2016 in a nearby tertiary care hospital but it was performed in the operation theatre because of concerns about autonomic dysreflexia. On discharge, the patient was informed to contact her General Practitioner if she developed symptoms of urine infection. The patient did not receive any literature with regard to side effects of muscle weakness following bladder wall injection of Abobotulinum toxin A. About 2.5 weeks later, this patient noticed weakness of her arms. Muscle weakness did not happen abruptly; muscle weakness occurred gradually after 2.5 weeks following Abobotulinum toxin A injection. When she was lifted on to the platform for a bath, she could not lie down herself or lift herself up. She could not move forwards or backwards without support. After Abobotulinum toxin A injection, she could not lift herself for pressure relief. She could not transfer herself using a sliding board whereas she was doing transfers using a sliding board very easily prior to Abobotulinum toxin A injection. She could not put her arm in to her coat. She experienced worsened balance and felt unstable when she leaned forward. She did not develop difficulty in swallowing. In about 6 weeks, she regained her muscle strength.
Approximately 3 weeks after Abobotulinum toxin A injection, the patient had a close family bereavement, which had affected her emotionally. The patient’s muscle weakness having lasted for a short period of time and having no knowledge of the full side effects or risks of botulinum toxin, and her mind preoccupied by the recent bereavement, the patient made no causal association between the muscle weakness and the bladder wall injection of Abobotulinum toxin A even though the muscle weakness occurred soon after Abobotulinum toxin A therapy. During the follow-up appointment in October 2016, the length of time that had passed after the occurrence of muscle weakness also contributed to the failure to communicate this side effect back to the physician.
In October 2016, the patient was noted not to get any significant urgency or urinary leak, however, she started getting symptoms of urinary tract infections and she was prescribed nitrofurantoin 50 mg nocte. In February 2017 the symptoms of neurogenic over-activity had returned; flexible cystoscopy and intra-vesical injection of botulinum toxin were planned under local anaesthesia but for an Anaesthetist to be on standby in case this patient developed autonomic dysreflexia. Now in hindsight, the patient felt that had she been aware of the possibility of generalised muscle weakness after botulinum toxin injection, she would not have consented to the second bladder wall injection of Abobotulinum toxin A; again with the hindsight, she felt she would have requested for an immediate follow-up appointment with her consultant urologist.
In June 2017, Abobotulinum toxin A, 1000 units, was injected into the urinary bladder in the same hospital where the first botulinum toxin injection was administered. The consent form used for both procedures did not contain any warning regarding potential risk of distant spread of muscle weakness or a ‘Black Box Warning’ as stated by FDA. The black box warning states ‘Post-marketing safety data from approved botulinum toxins suggest that botulinum toxin effects may, in some cases, be observed beyond the site of local injection. The symptoms are consistent with the mechanism of action of botulinum toxin and may include asthenia, generalised muscle weakness, diplopia, ptosis, dysphagia, dysphonia, dysarthria, urinary incontinence and breathing difficulties. These symptoms have been reported hours to weeks after injection. Swallowing and breathing difficulties can be life threatening and there have been reports of death related to spread of toxin effects.’ []
Weakness of muscles came quicker after the second injection of Abobotulinum toxin A. Ten days after Abobotulinum toxin A injection, this patient could not sit up or lie down independently. Weakness of arms as well as the hands was noticeable; she was easily fatigued. Muscle weakness was severe in her arms, hands and trunk. When she tried to put her coat on, she did not have the strength to push her arm through the coat. She could not open a blister tablet pack, was unable to lift lightweight objects and she was struggling to hold a pen. Prior to Abobotulinum toxin A injection, she could lift herself so that her partner could apply soap and wash her buttocks. Following Abobotulinum toxin A injection, she could not lift herself and her partner was unable to clean her buttocks. The patient’s respiratory muscles were affected; she could not project her voice as strongly as she did prior to Abobotulinum toxin A injection. When she was sat up, she was struggling to call her caregivers. She was not able to shout for her partner across the road when they went out. She could not cough as effectively as she did before Abobotulinum toxin A injection. She had no truncal balance; she was very concerned that she might fall out of her wheel chair. The patient’s blood pressure was severely affected; blood pressure would become very low when first sitting up in the morning. Patient suffered with light-headedness and on one occasion, passed out. Prior to Abobotulinum toxin A injection, she only drank 300 ml of milk with morning medication when sat up. Now she needed caregiver support to sit up and required to drink an additional 500–750 ml of water before blood pressure would become stable.
In October 2017, this patient felt that she had not regained strength in her triceps fully. The weakness of her hands also persisted. She was still unable to lift herself after she was laid in the bath. When she was going down the drive in her wheelchair, she was losing control of her wheelchair. When she was holding a pen, she could not continue to grip the pen after writing one sentence. She could not apply enough pressure to write legibly. She was unable to lift herself and move to the left for car transfers. She could not position herself on the chair. She was still unable to do pressure lifts. She was no longer self-caring; everything was being done for her.
In February 2018, the patient had regained some muscle strength in her arms; however, daily exercise of biceps and triceps was still on going to facilitate possible improvement to the level which she had prior to Abobotulinum toxin A injection. Patient’s pincer grip and strength in both hands was still weak with slower recovery. Self-propelling of wheelchair was still difficult, particularly on uneven surfaces. She was not confident to push her wheelchair alone when outdoors. The patient felt it would take longer for her to regain dexterity and strength in her hands and fingers to the pre Abobotulinum toxin A injection status.
After Abobotulinum toxin A injection, this patient could not stop oxybutynin. She required to continue oxybutynin 5 mg twice a day by mouth and applied oxybutynin skin patch changing it every third day.
Following Abobotulinum toxin A injection, this patient noticed that she had to take an increased dose of senna to maintain regular bowel movements. Before Abobotulinum toxin A injection, she was taking 18 ml of senna; after Abobotulinum toxin A injection, this patient had to increase the dose of senna to 30 ml.
In the timeline from the first appointment in May 2016 to her final appointment in October 2017, the patient had been transferred from one consultant to another consultant, and had seen four different doctors. In October 2017, the physician in a tertiary care hospital advised the patient not to have any further injection of botuinum toxin, as was apparently clear that the patient developed upper limb weakness and respiratory problems following Abobotulinum toxin A injection on both occasions.
During subsequent visit to the spinal unit, this patient was advised to continue oxybutynin tablets, transdermal oxybutynin patches and regular intermittent catheterisations.
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pmc-6234438-1
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We report the case of a 4-year-old girl, who was diagnosed with NF type 1 at the age of 14 days. Her mother and her older brother also suffer from NF type 1. During the annual follow-up at the age of 2.5 years, she had office BP levels below the 90th percentile (p.c.) for age, sex, and height (105/59 mmHg, 98 bpm). A routine ambulatory blood pressure monitoring (ABPM) showed a nondipping profile with normal mean BP levels (mean 24 h BP 107/61 mmHg, mean 24 h HR 99 bpm) ().
One year later, at the age of 4 years, her office BP was greater than the 95th p.c., and a difference of 20 mm Hg between upper and lower extremities was documented (). Clinical examination also showed a systolic murmur of 3/6 with punctum maximum on the Erb point, weak femoral and pedal pulses, and absent tibial posterior pulses on both sides. Other clinical findings were multiple cafe au lait signs on her whole body and underdevelopment of the left leg. ABPM revealed daytime and nighttime hypertension (). The patient underwent the necessary laboratory and imaging examinations to diagnose the cause of hypertension, and she commenced on valsartan with the addition of felodipine because of inadequate BP control (). Laboratory exams showed normal renal function. Renal ultrasound demonstrated a right kidney length of 7.1 cm and a left kidney length of 8.5 cm. Echocardiography did not reveal left ventricular hypertrophy or any evidence of other cardiac anatomical or functional abnormalities. Fundoscopy was normal. Measurement of cf-PWV (SpygmoCor, AtCor Medical) showed increased arterial stiffness (). Magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) imaging of the brain, spinal column, and abdomen and X-ray examination of all long bones revealed multiple brain hamartomas and one neurinoma at the 10th vertebra of the thoracic spine. Magnetic resonance angiography of the aorta and renal arteries showed abdominal aortic stenosis (lumen diameter 0.30 cm) and inability of imaging the arise of right renal artery. A subsequent computed tomography angiography (CTA) documented severe segmental aortic stenosis arising before the origin of the upper mesenteric artery and including the origin of renal arteries. Renal arteries also presented ostial stenosis (). The patient underwent percutaneous transluminal angioplasty (PTA) in the abdominal aorta, while direct dilatation of renal arteries was not possible to perform due to small diameter of renal arteries. There was deterioration in renal function after angioplasty due to contrast-induced acute renal injury accompanied by deterioration of BP levels. A DTPA (99mTc-diethylenetriaminepentaacetic acid) renal scan showed 25.8% left kidney contribution in the total renal function.
On her follow-up visit 4 months after PTA, there was no difference in BP between the upper and lower extremities, and pulses were present on both sides. Office BP levels presented a decrease but remained greater than the 95th percentile. Moreover, 24 h BP increased and cf-PWV was further increased, despite pharmacological treatment with amlodipine, furosemide, and atenolol. Clonidine was subsequently added to treatment. On the other hand, renal function was significantly improved. Both MRA and CTA showed a moderate improvement of the anatomical structures with abdominal aorta stenosis ranging between 0.35 and 0.5 cm, while other findings remained constant.
During her next follow-up visit, 8 months after PTA, renal function was further improved, and the patient presented significant reduction of ambulatory BP levels and a reduction in cf-PWV values despite sustained office BP elevation (). However, at 12 months after angioplasty, ambulatory BP levels were again increased at preangioplasty levels. In contrast, cf-PWV was further decreased, while MRA findings of the aorta and renal arteries remained unchangeable.
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pmc-6234446-1
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The patient was a 70-year-old man who presented with fatigue and loss of appetite. He had a medical history of diabetes mellitus (DM) and hypertension and was receiving pharmacotherapy for both diseases. Laboratory examination showed thrombocytopenia (83,000/µL; normal range: 140,000–400,000/µL) and an elevated concentration of lactate dehydrogenase (LDH) (464 IU/L; normal range: 119–229 IU/L). DM was poorly controlled (hemoglobin A1c: 8.8%; normal range: 4.6–6.2%). Chest X-ray and computed tomography (CT) showed consolidation and surrounding ground-glass shadows in both lungs (), and transbronchial lung biopsy was therefore performed. Histopathological analysis revealed diffuse proliferation of medium-sized lymphoid cells. Tumor cells showed expressions of CD3, CD4, CD56, TIA-1, and granzyme B and in situ hybridization for Epstein–Barr virus- (EBV-) encoded small RNA (EBER-ISH), but absence of CD5, CD8, CD10, and CD20, leading to the diagnosis of ENKL (Figures –). Otolaryngological examination was performed on a precautionary basis, but no abnormalities of the nasal mucosa were found. Positron emission tomography (PET)/CT was performed to search for other lesions, revealing abnormal uptake in the stomach in addition to the lung lesions (). Gastroscopy showed an ulcerative lesion () that was biopsied. Histopathological analysis showed diffuse proliferation of large lymphoid cells infiltrating under the mucosa. Tumor cells lacked expressions of CD3, CD5, CD10, CD56, bcl2, bcl6, and EBER-ISH and positive results for CD20, CD79a, and MUM1, leading to the diagnosis of DLBCL (nongerminal center B-cell-like type) (Figures –). Negative results were obtained for Helicobacter pylori. Bone marrow aspiration showed no invasion of tumor cells. The serous ferritin level was 2,260 ng/mL (normal range: 39.4–340 ng/mL). Antibodies to EBV showed a prior infection pattern, but EBV-DNA was elevated to 1.7 × 105 copies/106 cells and the concentration of soluble interleukin 2 receptor was 3,760 IU/mL (normal range: 145–519 IU/mL). We diagnosed composite lymphoma comprising ENKL and DLBCL. Chemotherapy was started with dexamethasone, etoposide, ifosfamide, and carboplatin (DeVIC) plus rituximab. During the clinical course, bone marrow was strongly suppressed and febrile neutropenia occurred. Piperacillin/tazobactam and granulocyte-colony stimulating factors were used, and platelet transfusions were necessary to address severe thrombocytopenia. After two courses of chemotherapy, gastrointestinal endoscopy showed shrinkage of the ulcerative lesion () and elimination of lymphoid cells in the biopsy. On the other hand, lung lesions did not show any improvement, and the chemotherapy regimen was therefore changed. After one course of chemotherapy with gemcitabine, dexamethasone, and cisplatin (GDP), the disease remained progressive and dyspnea appeared. Best supportive care was initiated, and the patient died 3 months after diagnosis.
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pmc-6234448-1
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This is a 24-year-old autistic nonverbal male who was brought into the emergency room (ER) by his mother for increasing lethargy and frequent urination for a few days. Prior to admission, he had several episodes of nonbloody vomiting, but mother denies any history of fall, prolonged immobilization, medication overdose, and increased physical activities. On admission, the patient's vitals were stable. Physical examination was significant for confusion, dehydration, and lethargy. Laboratory investigation in the emergency department revealed () hypernatremia, highly elevated blood glucose level, elevated serum creatinine of 5.29 mg/dl (reference range 0.61–1.24 mg/dl), low bicarbonate level 13 mmol/L (reference range 24–31 mmol/L), hyperphosphatemia, and high anion gap metabolic acidosis on arterial blood gas analysis. The patient was found to be in a hyperosmolar hyperglycemic state with a serum osmolality of 395 mmol/kg (reference range 275–295 mmol/kg). Serum acetone level was large (reference range negative). Urinalysis was significant for ketones, proteinuria, high glucose level, and dark tea-colored urine. Urine blood was large; however, only few red blood cells were present. In the ER, patient was aggressively hydrated with intravenous (IV) normal saline boluses and kept on maintenance fluid with replacement of electrolytes. A computed tomography (CT) of the abdomen pelvis was consistent with pancreatitis although his amylase and lipase was normal. The patient was admitted to the intensive care unit (ICU) and was treated for DKA with standardized protocol. After resolving of acidosis, the patient was transitioned to subcutaneous insulin and started on a diabetic diet. The patient was diagnosed with diabetes type 2 on this admission. Because of new onset diabetes, glutamic acid decarboxylase was performed, which returned less then <5 IU/mL (reference range: 0.0 to 5.0, unit: IU/mL). The patient's hemoglobin A1c was elevated at 12.9% (reference range: 4.5–6.0%). The patient's acute kidney injury was attributed to dehydration with a fractional excretion of sodium (FENa) less than 1% suggestive of prerenal. His kidney function was expected to improve with volume replacement and DKA treatment. His renal function progressively worsened and became anuric and was started on hemodialysis. Because of persistent poor renal function, a creatinine phosphokinase (CPK) level was sent, which returned highly elevated at 129,940 (IU/L) (reference range 22–232 IU/L). He was maintained on a high rate of intravenous normal saline and hemodialysis. In the next two days, his CPK level started to trend downward and became normal upon on discharge. The patient required 2 months intermittent hemodialysis and developed chronic kidney disease stage 2 with a glomerular filtration rate of 80.
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pmc-6234537-1
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A 32-year-old Tamil woman presented with heavy menstrual bleeding of 4 months’ duration. She had no previous gynecological issues and had delivered two children vaginally. Her past medical history and family history were unremarkable. There were no psychosocial stresses. On examination she was pale, had no palpable lymph nodes, no hepatosplenomegaly, a large pelvic mass, and three firm vaginal nodules. Her hemoglobin was 5.2 g/dl and she had a white blood cell count of 9100/mm3 with 50% lymphocytes and platelets of 487,000/mm3. Blood picture showed evidence of microcytic anemia. A pelvic ultrasound suggested a fibroid uterus with two large pedunculated fibroids. Following preoperative optimization, dilatation and curettage and biopsy of the vaginal nodules were done. Histology revealed proliferative phase endometrium. The vaginal nodules showed lymphoid tissue.
A week later, she developed fever, features of an acute abdomen, and ascites. Her white blood cell count had risen to 36,000/mm3 with predominant lymphocytes. An emergency laparotomy was done which revealed two solid ovarian masses (Fig. ), gross ascites, omental deposits, enlarged mesenteric lymph nodes, and a bulky uterus with thickened infundibulopelvic pedicles. A bilateral oophorectomy was done for histological diagnosis and to relieve bowel compression. A hysterectomy was not done as there was pelvic side wall involvement. Her lactate dehydrogenase (LDH) was 2250 IU/L with normal serum β-human chorionic gonadotropin (β-hCG), alpha-fetoprotein (AFP), and cancer antigen-125 (CA-125) levels. Histology revealed a diffuse large B cell lymphoma. As her general condition was deteriorating, she was started on the cyclophosphamide, adriamycin, vincristine, and prednisolone (CHOP) chemotherapy regimen while awaiting staging investigations. She had a dramatic clinical improvement with the first cycle of chemotherapy.
A bone marrow biopsy revealed 80% infiltration with lymphoid cells. Imaging showed enlarged lymph nodes above and below her diaphragm, ascites, and hepatosplenomegaly. Immunohistochemistry revealed focal CD20 staining and nuclear positivity for terminal deoxynucleotidyl transferase (TdT) and scattered CD3 positivity which suggested a B cell lymphoblastic lymphoma. Considering the blood count with bone marrow findings, a diagnosis of lymphoblastic lymphoma/leukemia was made according to World Health Organization (WHO) classification []. Cerebrospinal fluid (CSF) cytology was normal. Cytogenetics was not done due to financial constraints.
She was later commenced on UKALL XII Trial protocol containing prednisolone, vincristine, daunorubicin, asparaginase, and intrathecally administered methotrexate and was in remission at the end of phase 1 induction chemotherapy. However she developed hepatotoxicity which precluded continuation of chemotherapy and eventually she died. A timeline to show disease progression is shown in Fig. .
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pmc-6234673-1
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A 9-year-old boy complained of malaise, just a few minutes after his first summer swim in the sea; soon after, he presented generalized urticaria, dyspnea, conjunctival hyperemia, blurred vision and faintness. When first aid arrived, since anaphylactic shock was suspected, intramuscular steroids, intravenous antihistamine and nebulized salbutamol were administered, with rapid improvement on the part of the patient. The only thing of note in the child’s medical history was allergy to dust mite, and no other allergies were reported. There was no evidence of any insect bite or drug ingestion; an hour before the swim, the child had eaten his usual breakfast, with hot chocolate. Apparently, there was not contact with fish during the swim. The child had never complained of similar symptoms before and had never had urticaria after contact with water, be it seawater or tap water. No familiarity for allergic disease or chronic urticaria was reported. The child was referred to the local Allergy Department and in order to identify the offender, skin tests and specific IgE assays were performed. In detail, they tested allergy to milk, due the history of milk intake before the appearance of symptoms, and to insect venom and fish, because of the possibility of contact with insects and fish during the bath; all the tests were negative. Although the patient developed no symptoms on contact with tap water, an aquagenic urticaria was suspected, but the specific test was negative. Finally, a cold urticaria was suspected but the cold stimulation test (CST) was negative too. Given the severity of the reaction, prophylactic antihistamine therapy was commenced, but in spite of this, throughout the summer the patient continued to develop wheals all over his body after every swim in the sea (Fig. ), even in places where there had been no direct contact between the skin and the water. The child then came to our attention, at the Burlo Garofalo Institute for Maternal and Child Health in Trieste (Italy), the referral centre for allergic diseases in the north-east of Italy. There, based on the child’s clinical history, a diagnosis of an atypical form of cold urticaria (ACU) was formulated. The specific diagnostic test of ACU involves keeping the lightly clothed patient in a cold room (at a temperature of 4 °C) for 30 min; in our case, it was avoided because of the past patient’s severe systemic reaction. In any case, the boy’s history was fairly characteristic enough to confirm the diagnosis of this rare and often unrecognised chronic physical urticaria: typical diagnostic features of ACU are in fact the appearance of wheals after exposure to various sources of cold (such as seawater at the beginning of summer), also in areas not directly in contact with water and the negativity of the CST. Antihistamine therapy was continued for the whole summer with fair control of symptoms and self-injectable epinephrine was prescribed but the child has never used it.
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pmc-6234700-1
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This is a case of a 25 years old Malay girl with learning disability and no significant past medical history, who started noticing a sacral mass since August 2015. The mass was painless and gradually increasing in size. The family members of this patient brought her to a traditional healer. They did not seek any medical treatment until late 2017. By this time, the mass over the sacrum was extremely large. Family members claimed the mass was preventing the patient from lying down flat supine. The patient was also unable to ambulate for the past 2 years. Hence, she was bedbound most of the time. It was difficult for her to sit on the wheelchair. She also felt tired to move because the mass was quite heavy. The family members claimed when the patient was lying down flat, she had to flex her hips and knees to achieve a more comfortable position. In addition, she often slept either in prone position or in supine with multiple pillows below her body. The mother also claimed over the last 2 months, the patient’s body had been getting thinner despite her physical weight was increasing due to the increase in size of the sacral mass. The patient had been passing stool and urine in pampers. There was no past medical history and no family history of cancer. Socially, the patient lived with her mother and siblings. The mother was the main care taker. Her father passed away 10 years ago because of heart attack. The patient previously attended a special needs school, but she stopped going to school since 2015 after developing the sacral mass.
This patient was managed in the Southern Region referral centre for Orthopaedic Oncology in Malaysia. On clinical examination in the Orthopaedic Oncology ward, the patient appeared cachexic, she had slightly pale conjunctiva, but she was not dysmorphic. Vital signs were Blood Pressure 142/90, Pulse Rate 98 beats per minute and Temperature 37 degrees Celsius. There was a large mass 40 cm × 30 cm × 20 cm over the sacrum. The mass was firm to hard in consistency and involved both buttocks and the gluteal fold (Fig. ). Dilated veins were noted under the skin overlying the sacral mass. Neurological exam of bilateral lower limb was normal. However, there was generalized wasting of all muscles over the bilateral lower limb. Anal tone was intact.
Laboratory investigations taken were unremarkable. Computed Tomography of the Pelvis showed a large destructive sacrococcygeal mass measuring 43 cm × 38 cm × 27 cm with extension into the presacral space resulting in anterior displacement of the rectum, urinary bladder and uterus and posterior extension into the dorsal soft tissue with involvement of the gluteus, piriformis, and left erector spinae muscles (Figs. and ). Superior margin of the sacral bone involvement was up to S2. The mass was predominantly of fluid density with internal enhancing septation and calcifications which suggested primary chordoma more likely (Figs. and ). Magnetic Resonance Imaging done showed similar findings. Skeletal Survey Radiograph did not show any distant metastasis. A Trucut biopsy of the mass was done. Histopathological analysis showed tumour cells with “physaliphorous cells” positive for pancytokeratin, EMA, Vimentin and S-100 immunohistochemistry stainings with minimal mitotic figures and mild nuclear pleomorphism (Fig. ). Brachyury immunohistochemistry staining was not available in our centre. However, the clinical history, morphology of tumour on microscopy and immunohistochemistry staining available were consistent sacral chordoma.
The diagnosis of Sacral Chordoma was confirmed. Multidisciplinary team discussion done among Orthopaedic Oncology, General Surgery, Obstetrics and Gynaecology, Blood Bank, Anaesthetic and Plastic Surgery teams. A family conference was done. The family’s aim was for removal of the sacral mass to allow the patient lie supine on bed and sit on the wheelchair.
Subsequently, the patient undergone Wide Resection and En Bloc Sacrectomy. The Posterior-Only Approach was used with a “Mercedes Star” 3 limbed incision. Duration of surgery was 8 h. The patient was supported with blood products transfusion during surgery. Intraoperatively, the sacral tumour had eroded the sacral bone from S2 to S5 (Figs. , and ). Sacrectomy was done at the level of S2. Sacral nerve roots S2-S5 were all infiltrated by the mass and therefore were unable to be preserved. The mass and surrounding gluteal muscles invaded by the tumour were also all resected. All resection margins were less than 1 mm from the tumour. Primary closure was done without any distant or local flap as per consultation with Plastic Surgery team. The tumour weight was 25 kg (Figs. , and ). Post operatively, the patient was monitored in Intensive Care Unit for 3 days. The patient developed neurogenic bowel and bladder post sacrectomy requiring enema and long-term urinary catheter. In addition, the post-operative course was complicated by wound breakdown and surgical site infection requiring wound debridement. Dressing was done as per local protocol until wound bed granulating well. Split Skin Graft was done about 3 months post wide resection once the tissue culture results were free of significant infection.
The patient also required extensive rehabilitation for transfer, ambulation and bowel and bladder care. Rehabilitation was difficult because the patient had learning disability and she had been habitually keeping her hips and knees flexed because of the sacral tumour for the past 2 years. During the last review 5 months post operatively, patient was able to sit on the wheelchair comfortably. The surgical wound was healing well with good uptake of the Split Skin Graft (Fig. ).
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pmc-6234701-1
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A 51-year-old female with no subjective symptoms was referred to our hospital with a left intrathoracic mass that was discovered during a medical checkup. Computed tomography (CT) revealed a neoplastic tumor (43 × 48 × 13 mm) with extrapleural signs, located in the dorsal portion of the left thoracic cavity (Fig. a). The tumor demonstrated mixed and heterogeneous absorption, which indicated fat and soft tissue. Positron emission tomography (PET) revealed no significant uptake by the tumor. These findings suggested that the mass was a benign lipomatous tumor or lipoma with bleeding originating from the parietal pleura. However, the possibility of a malignant liposarcoma could not be excluded; therefore, we decided to excise the lesion to determine its pathological diagnosis and subsequent treatment.
The patient was intubated using a double-lumen endobronchial tube under general anesthesia and was placed in the right lateral position. After initiating one-lung ventilation, three thoracoscopic ports were placed on the left chest wall, demonstrating a pedunculated mass hanging from the parietal pleura, without attachment to the lung (Fig. b). The parietal pleura was incised near the tumor stalk, which allowed easy dissection of the tumor form the chest wall and extirpation via complete thoracoscopic surgery.
The excised specimen was a yellowish-white, soft elastic tumor with a thin fibrous coating (Fig. c). Histopathological examination revealed a fibrous component in the tumor as well as dense adipocyte growth (Fig. d). Based on these findings, the tumor was diagnosed as a fibrolipoma. The patient experienced no postoperative complications and was discharged on postoperative day 5. After 2 postoperative years, the patient is alive without recurrence.
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pmc-6234783-1
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This boy was born at 37+ 2 weeks of gestation from a 30-year-old mother. He was the first child of non-consanguineous and healthy Caucasian parents. The patient’s mother received prenatal care during pregnancy. At 12 weeks, Doppler ultrasound (US) examination revealed increased resistance in the uterine arteries, and salicylic acid (100 mg daily) was prescribed. From the 23rd week, several Doppler US scans showed intrauterine growth retardation with persistent notching in the right uterine artery and increased resistance in the left. No scan revealed a malformation. Labour was spontaneous and the boy was born by vaginal delivery using Thierry’s spatula because of abnormal foetal heart rate. Apgar scores were 4, 7 and 8 at 1, 5 and 10 min, respectively; arterial cord blood pH was 7.22 and cord lactate was 5.1 mmol/L. Birth weight was 2045 g (0.6th centile, according to customized French curves), length was 51 cm (91th centile), and head circumference was 32 cm (10th centile). The placenta was hypotrophic (280 g), with peripheral insertion of the cord and signs of maternal vascular hypoperfusion, but no lesion of decidual arteriopathy at pathological examination.
Respiratory distress, including suprasternal tugging and stridor, was observed immediately following birth. The neonate was bagged with air for a few minutes and then supported with nasal continuous positive airway pressure (CPAP). Prolonged apnoea associated with bradycardia required caffeine from the first postnatal day.
The first series of exams showed normal brain US and normal serum electrolytes with calcium. Flexible fibreoptic laryngoscopy (FFL) performed on day 2 revealed BVCP in the adducted position, causing severe airway obstruction and prompting transfer to a medical and surgical neonatal intensive care unit on day 3.
On admission, clinical examination revealed a wide anterior fontanel, enlarged coronal sutures, normal temperature, and persistent respiratory distress despite CPAP. Facial and ocular motricity were apparently normal, and the sucking reflex was present. The newborn was intubated a few hours later for worsening respiratory distress and severe apnoea. Rigid laryngotracheal endoscopy performed on day 4 under general anaesthesia showed neither malformation nor other obstruction below the glottis. Chest X-ray and abdominal and cardiac US were normal.
Neuroradiological investigations were performed between day 4 and day 15. The first brain MRI showed a deformation of the bulbo-medullary junction with an arched aspect in the anteroposterior direction slightly deflected to the left. Enlarged subarachnoid spaces and foramen magnum surrounded this junction. Cortical structures were normal, myelination was age-appropriate, and the pituitary axis and corpus callosum were normal (Fig. ). Additional investigations were conducted, with MRI and CT focused on the cervico-occipital hinge. MRI showed a unilateral cavitated lesion, with discontinuity between the upper pons and the medulla oblongata. CT revealed vertebral anomalies, with hypoplasia of the right posterior hemiarches of C1-C2, hypoplasia of the right exo-occipital bone and a small clivus (Fig. ). On day 15, MR angiography showed the absence of the distal right vertebral artery (Fig. ). MRI with fibre tracking, using diffusion tensor imaging, confirmed hemisection of the right lateral and median sensorimotor fascicles (Fig. ). Brainstem auditory evoked potentials and fundus examination were normal.
Patient management included a left unilateral endoscopic laser cordotomy on day 7 in order to proceed to extubation on day 8. The procedure was unsuccessful despite corticosteroid therapy and CPAP, and the infant was reintubated after 3 h due to the reappearance of stridor and major signs of respiratory distress. Mechanical ventilation was maintained until the end of the stay. The severity of this situation prompted a multidisciplinary ethics consultation and the decision was made to limit care to the infant’s comfort. The parents were informed and agreed with this decision, given the very poor respiratory and neurosensory prognosis. The infant died at the age of 4 weeks. An autopsy was not performed. Array comparative genomic hybridization revealed no abnormality.
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pmc-6234785-1
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A 39-year-old Greek woman, a nurse in our military hospital, presented to our department with repeated symptoms of flatulence and epigastric discomfort over a few months. Her past medical, social, environmental, and family history was unremarkable for any illness or causative factor. She was not on any medication, she did not smoke tobacco or consume alcohol, and she was afebrile at the time of admission. Her neurological examination was normal; her blood pressure was 126/84 mmHg, her pulses were regular at 75–80 beats/minute, and her temperature was 36.8 °C. Laboratory testing revealed the following results that are shown in Table : a rise in erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) inflammation markers as well as a rise in her lactate dehydrogenase (LDH) tissue necrosis index (Table ).
A computed tomography (CT) scan showed a large mass measuring approximately 24 cm in its greatest dimension (23.7 cm × 16.5 cm × 11.5 cm) that originated from her right adrenal gland and occupied her right abdomen, while compressing her right hepatic lobe and her inferior vena cava, and it was in contact with the right perirenal fascia of Gerota (Figs. and ).
A functional adrenal work-up was performed and included: measurement of serum aldosterone, potassium, renin, and adrenocorticotrophic hormone levels; a dexamethasone suppression test; and measurement of 24-hour urinary metanephrine levels. All results were within the reference ranges. A fine-needle core biopsy revealed ACC.
A metastatic work-up included CT scans of her head and chest and a bone scan and they were negative for metastases. During laparotomy the giant tumor was removed completely with its own capsule, without the need for excision of adherent organs as there were no infiltrations.
Postoperative pathology results confirmed the diagnosis of ACC and no further adjuvant treatment was applied to our patient (Fig. ). Her postoperative course has been uneventful for 1.5 years.
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pmc-6234785-2
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A 67-year-old Greek woman, a retired high-school teacher, presented to our department after an evaluation for fatigue, mass effect, epigastric discomfort in liver cirrhosis, and hypothyroidism. Her past medical history was also remarkable for arterial hypertension. She was on double anti-hypertensive medication and she was also receiving levothyroxine 100 μG once daily. She was a heavy tobacco smoker (>1pack/day) for 35 years and a social alcohol consumer. She was afebrile at the time of admission. Her neurological examination was normal; her blood pressure was 145/97 mmHg, her pulses were 95 beats/minute, and her temperature was 36.4 °C. Her mother died from breast cancer.
Laboratory testing revealed results that are shown in Table . A CT scan revealed a large invasive mass in the anatomical area of her left adrenal gland, well circumscribed, measuring 7 × 7 × 9 cm; it extended to the upper pole of her left kidney and the inner hilum of her spleen without infiltration of the above organs, which showed marked heterogeneous enhancement after intravenous infusion of a contrast agent, which posed a differential diagnosis problem with possible pheochromocytoma (Fig. ).
Further laboratory testing of post-prandial plasma cortisol and plasma testosterone levels gave normal results, mimicking a nonfunctional left ACC. Elective open adrenalectomy was scheduled without any complications and the postoperative pathology record was consistent with a pseudocyst, without evidence of malignancy (Figs. and ). She has had an uneventful course 1 year postoperatively.
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pmc-6235633-1
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Past medical history and symptomatology
A 72-year-old white male with a past medical history significant for hypertension, dyslipidemia, coronary artery disease status post-coronary artery bypass grafting, and chronic heavy smoking presented with a two decade history of seizure disorder due to an undefined brain lesion. At presentation, the patient had new deficits including slurred speech, ataxia, weakness, fatigue, and altered mental status. He had also lost weight over several months. He and his family denied any change in his seizures, new headaches, nausea, vomiting, or sensory changes. His initial head-CT showed a right temporal/frontal lobe mass with vasogenic edema, intralesional calcifications and 5 to 6 mm of midline shift with effacement of the temporal lobe sulci. Further investigation via magnetic resonance imaging (MRI) (Figures -) revealed what appeared to be multiple different lesion types including both extramedullary and intramedullary masses. The extramedullary portion of the lesion was hypointense on T1 non-contrast MRI as well as T1 post-contrast MRI (Figure ) and appeared to be abutting the dura of the antero-medial middle temporal fossa. The intra-axial mass was hyperintense on T1 non-contrast imaging and showed diffusion restriction at its periphery with post-gadolinium enhancement of the immediately adjacent brain tissue. Finally, the proximal middle cerebral artery was coursing directly between these two areas of the lesion.
Surgical procedure
After a thorough discussion with the patient and family, the patient underwent right frontotemporal craniotomy with image guidance in order to obtain diagnosis and to resect the lesion. A pterional craniotomy large enough to expose the sylvian fissure and the adjacent frontal and temporal cortices was created and microscopic dissection was completed to open the sylvian fissure widely and to expose the insular cortex and the adjacent frontal and temporal areas. The extramedullary component of the mass was firm and partially calcified. This could not be mobilized away from the middle cerebral artery (MCA) trunk and had a clear border outside the brain tissue. The surrounding brain tissue was abnormally soft and showed macroscopic evidence of necrosis with traversing thrombosed vessels. The extramedullary lesion was opened sharply with microscissors and within this lesion, there appeared to be densely packed old hemorrhage and hemosiderin deposition. A portion of the central contents of the lesion was sampled and sent for pathology as well as a piece of the lesion wall. The findings of old clotted blood within this lesion and dense adherence to the MCA vessels raised clinical concerns for a partially thrombosed aneurysm and it was decided to allow the remainder of the lesion to remain intact. Subsequently, surrounding brain tissue was removed and intra-operative pathological analysis from this tissue resulted in the diagnosis of a high-grade glioma. The suspected GBM was then debulked to the edge of its enhancing border.
Histopathological findings
The first biopsy of the right insular tumor (Figures -) showed structures consistent with a dermoid cyst. This biopsy included old blood/hemorrhage with keratinaceous debris. There was no evidence of organization and no epithelium was identified. Figure depicts a mass with thin lining of squamous cells that is filled with squamous debris indicating the presence of dermoid cyst. Squamous cell and debris are pancytokeratin positive (Figure ).
The second biopsy (Figures -) area taken from the right insular tumor demonstrated features consistent with a GBM including increased cellularity, nuclear pleomorphism, necrosis and vascular proliferation. It shows highly cellular and irregular malignant cells which are positive for Glial fibrillary acidic protein (GFAP) (Figure ), with necrosis indicating the presence of GBM.
Finally, the third biopsy (Figure ) from the extramedullary portion of the lesion adjacent to the middle cerebral artery was determined to be consistent with an aneurysm with features including dense collagenous tissue with calcification and remodeling into bony trabeculae. In this figure, we can see all three structures next to one another.
In order to examine the presence of inflammatory cells within the lesion, specific stains were applied to the aforementioned specimens. Figure and Figure depict a slide stained for CD 68 to examine for macrophages. As shown, the stain was positive for CD 68, highlighting macrophages within the cyst wall and the tumor, indicating the presence of inflammatory cells.
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pmc-6235635-1
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A 23-year-old African American man with a history of bipolar disorder presented to the Comprehensive Psychiatric Emergency Program with an altered mental state and copious vomiting. As per the emergency protocols, two large-bore (16 gauge) intravenous cannulas were placed, and a standard saline infusion was started to treat the patient’s descending blood pressure. The initial examination revealed a Glasgow Coma Scale Score of 15 (eyes, 4; verbal, 5; and motor, 6), and a quick neurological exam failed to reveal any deficits in the extremities. The patient exhibited normal muscle strength, deep tendon reflexes, and cranial nerve function. His gait could not be assessed due to fatigue and the emergency condition. Table presents the clinical laboratory values at admission.
The patient’s medication log stated he was currently taking VA 1500 mg daily, split into a 500-mg morning dose and a 1000-mg evening dose. His medical records revealed this VA dose was initiated four months prior after trials with other medications had failed due to adverse effects (diarrhea due to lithium). After starting VA, the patient was monitored via follow-up examinations in the clinic on a monthly basis. On his second-month follow-up visit, the patient reported concerns of weakness and fatigue. Laboratory tests revealed elevated ammonia and VA levels. At this time, the patient was diagnosed with VA-induced hyperammonemia. The VA treatment was stopped, and the patient was started on lactulose syrup and lamotrigine. We monitored the patient weekly. After his third weekly visit, he reported concerns of worsening of manic symptoms and severe bullae and rashes on his chest. The lamotrigine was stopped and manic symptoms recurred, leading to reinstating the VA treatment with weekly follow-up monitoring. His ammonia levels were elevated on all follow-up visits. A daily combination of lactulose syrup and levocarnitine was added to the treatment regimen in each weekly visit, but his ammonia levels continued to rise.
The patient presented with neurological symptoms with vomiting. Given his medical records, he was diagnosed with VA-induced hyperammonemic encephalopathy. His serum ammonia level was 68 µmol/L (reference range, 11 to 32 µmol/L). His serum VA level was within the reference range. After successful symptomatic control, the patient tested positive for an underlying genetic NAGSD. Therefore, we concluded this patient’s exaggerated response to VA in developing hyperammonemia was related to his underlying NAGSD. He was discharged from the inpatient facility with a daily dosing of 1500 mg VA and 800 mg carglumic acid.
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pmc-6235639-1
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A 67-year-old male chronic smoker with medical history significant for chronic obstructive pulmonary disease was admitted for imaging guided biopsy of a 1.2 cm left lower lobe lung nodule found recently on a computed tomography (CT) scan of the chest. The nodule was highly suspicious for primary lung malignancy. The patient was placed in a prone position and lung parenchyma in the posterior lateral left chest was visualized. Under CT guidance, a 19-gauge guide was advanced into the left lower lobe and two separate 20-gauge core biopsy specimens were obtained from the mass. There was no hemorrhage or immediate post procedure complication. However, towards the end of the procedure, the patient started complaining of sudden onset of chest pain and became unconscious. No palpable pulses were identified. A code blue was called and cardiopulmonary resuscitation was begun according to Advanced Cardiovascular Life Support guidelines. The patient subsequently demonstrated ventricular fibrillation which responded to defibrillation shock and epinephrine. The patient had three cycles of chest compressions, one dose of epinephrine, and a shock of 200 J. He was successfully resuscitated and intubated for mechanical ventilation. A CT scan of the chest was obtained immediately after the resuscitation and it demonstrated development of a small anechoic area in the left cardiac ventricle consistent with air embolus (Figure ).
The vital signs recorded at the time were as follows: blood pressure of 130/80 mmHg, pulse rate of 90 beats per minute, respiratory rate of 18 breaths per minute, and normal oxygen saturation of 92% on room air. The patient was admitted to the intensive care unit. Bedside transthoracic echocardiogram (TTE) did not reveal evidence of an air embolus. Additionally, no cardiac wall motion abnormalities were noted. The patient remained hemodynamically stable for 24 hours, and he was successfully weaned off from the mechanical ventilator next day and discharged home in a stable condition.
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pmc-6235640-1
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A four-week-old female born at term via unremarkable spontaneous vaginal delivery presented with a one-week history of irritability, poor feeding, and progressive somnolence. Prior to the onset of symptoms, her newborn period was unremarkable with good appetite, growth, voiding, stooling, and weight gain. Per parental report, she had a normal neurological exam in the nursery and at her newborn and two-week well child evaluations. She then began to have progressive feeding difficulty, becoming very irritable with feeds. She also became irritable with any attempted movement of her upper extremities. There were no fevers or hypothermia noted at home. Family and social histories were noncontributory.
On physical examination, she was afebrile with a heart rate of 130 beats/minute, respiratory rate of 40 breaths/minute, and irritable with any attempts at examination. Her head was normocephalic and her fontanel was soft and non-bulging. Her cardiac exam was without murmurs, her lungs were clear bilaterally, and her capillary refill was less than two seconds. Neurological examination was notable for absent bilateral Moro reflexes and decreased bilateral upper extremity grasp reflexes. Emergent computed tomography of her head was negative for an acute intracranial process. She was admitted to the inpatient ward where a lumbar puncture yielded slow-flowing, grossly xanthochromic fluid containing 132 nucleated cells with a normal differential. Ampicillin and cefotaxime were started. Blood, urine, and spinal fluid cultures were negative. She remained irritable and, over the next 12 hours, developed progressive hypotonia and areflexia of her bilateral lower extremities. Magnetic resonance imaging (MRI) of her brain was subsequently performed, which was also negative for acute intracranial pathology but demonstrated signal enhancement in the proximal cervical spinal cord (Figure ). Due to this finding, further imaging with cervical, thoracic, and lumbar MRI was completed, revealing a near holocord hemorrhagic, intramedullary mass (Figure ). Neurosurgery and oncology were urgently consulted and the patient was taken to the operating room for surgical resection on hospital day three. Pathology confirmed the diagnosis to be a congenital immature teratoma.
She was electively treated with carboplatin, etoposide, and ifosfamide chemotherapy along with radiation after surgical resection based on the Children's Oncology Group ACNS 1123 protocol. She completed this regimen after approximately 13 months and is currently in clinical remission. She has gross developmental delays; however, her motor function and residual neurological deficits are slowly improving with the help of early intervention.
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pmc-6235642-1
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A previously healthy 55-year-old man presented to the emergency department with an eight-day history of diarrhea. He reported that his diarrhea started shortly after eating a salad at a restaurant. He described his bowel movements as watery, nonbloody, and nonmucoid in appearance. The patient also complained of generalized fatigue, two episodes of vomiting, nausea, persistent low-grade fever, in addition to jaundice and dark urine. He reported that all his symptoms were acute, and denied any recent travel, hospitalizations, recent antibiotic intake, or unintentional weight loss.
On admission, he had a low-grade fever and was hemodynamically stable. Physical exam was relevant for pallor, jaundice, and mild diffuse abdominal tenderness. Workup revealed a hemoglobin level of 9.8 g/dL, a platelet count of 100,000 /cu.mm, a creatinine level of 3.5 mg/dL, an elevated blood urea nitrogen (BUN) level of 71 mg/dL, a direct bilirubin level of 2.2 mg/dL, a high lactate dehydrogenase (LDH) level of 879 IU/L (normal: 110-265 IU/L), a decreased haptoglobin and normal liver enzymes, prothrombin time (PT), partial thromboplastin time (PTT), and D-dimer levels. On peripheral blood smear, the patient was found to have a moderate number of schistocytes and helmet cells. The ADAM metallopeptidase with thrombospondin type 1 motif 13 (ADAMTS-13) activity and clusters of differentiation (CD) markers specific for paroxysmal nocturnal hemoglobinuria turned out negative. Stool studies were taken and qualitative multiplexed polymerase chain reaction (PCR) for a wide variety of bacteria, parasites, and viruses was done. Shiga-like toxin-producing E. coli (STEC) stx1/stx2 was detected in the stool specimen and led to the diagnosis.
During admission, the patient was adequately hydrated with intravenous (IV) fluids and daily laboratory parameters were monitored. On the second day of admission, the fever and diarrhea resolved completely. The patient’s hemoglobin level continued to drop for which he received one unit of packed red blood cells on admission day four. Clinically, the patient markedly improved and that was accompanied by improvement of all his laboratory parameters and he was discharged home on admission day five with scheduled follow up on an outpatient basis.
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pmc-6235643-1
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The patient, a 54-year-old male with a past medical history significant for coronary artery disease, hypertension, and hyperlipidemia, initially presented with complaints of dyspnea on exertion for several weeks. The patient reported experiencing recent weight gain, increased abdominal girth, and lower extremity edema. An inpatient echocardiogram showed moderate pericardial effusion with possible markers for tamponade. He underwent a pericardial window computed tomography (CT) scan that showed two incidental hypoattenuating foci in the liver, the largest measuring 2.2 cm in diameter (Figure ). There was no arterial enhancement within the lesions. Additional sub-centimeter hypo-attenuating foci were also noted but were too small to characterize by CT. A follow-up magnetic resonance imaging (MRI) scan of the abdomen and pelvis showed well-circumscribed T2 hyperintense lesions, which were hypo-enhancing to adjacent liver segments on post-contrast images (Figure ). At the time of admission, the patient’s labs were as follows: total bilirubin 0.8 mg/dL, direct bilirubin 0.2 mg/dL, aspartate aminotransferase (AST) of 16 U/L, alanine aminotransferase (ALT) of 25 U/L, alkaline phosphatase (ALP) of 94 U/L, and platelet count of 177 Thou/uL. The patient later underwent an outpatient ultrasound-guided liver biopsy of the right lobe mass. Cytology did not reveal evidence of malignancy. Of note, the patient did not have a history of liver disease and denied any history of heavy alcohol use, drug use, exposure to viral hepatitis, or occupational exposures.
Two months later, the patient returned to the hospital due to increasing abdominal pain. A CT scan of the abdomen and pelvis showed new lesions and nodules as well as evidence of hemoperitoneum presumed to be due to ruptured hepatic and splenic lesions. At the time, his laboratory findings showed: total bilirubin 3.7 mg/Dl, direct bilirubin 1.0 mg/Dl, AST 108 U/L, ALT 105 U/L, ALP 250 U/L, platelet count 29 Thou/uL, and lactic acid 4.6 mmol/L. A second liver biopsy was performed and pathology showed solid spindle cell proliferation. Immunohistochemical staining was positive for cluster of differentiation (CD)31, CD34, and Factor VIII, indicating likely HA (Figure ).
The patient was subsequently started on a cycle of gemcitabine. A follow-up MRI of the abdomen and pelvis two weeks later showed a progression of metastatic disease within the liver, spleen, spine, lung bases, and pericardium, with many of the metastases demonstrating signal characteristics consistent with interval hemorrhage (Figure ). The largest lesion was seen in the left lobe of the liver, causing mass effect and left-sided intrahepatic biliary ductal dilatation. The patient experienced multiple complications of his disease, including hepatic encephalopathy, anasarca, septic shock, and right pseudo-atrial aneurysm. Regrettably, the patient expired seven months following his initial diagnosis of metastatic HA.
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pmc-6235644-1
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An 18-year-old male presented to our institution in 2014 with headaches and vomiting for two weeks. A magnetic resonance image (MRI) of his brain showed a complex extra-axial dumbbell-shaped lesion with the epicenter in the left Meckel’s cave, extending anteriorly to the cavernous sinus and the cerebellopontine (CP) angle posteriorly with mass effect over the brainstem. He underwent a left retromastoid craniectomy and decompression of left CP angle lesion elsewhere. The histopathology was suggestive of malignant melanoma, and he was referred to our institution for further management.
His general physical examination revealed a deep gray-blue nevus over the left upper eyelid, extending to the frontal and temporal region. He had dysarthria, left upper motor neuron facial nerve palsy, and right hemiparesis with Grade 4 power in his right upper and lower limbs. The biopsy of the nevus over his left eyelid was reported as superficial, and the deep dermal dendritic melanocytosis with histological features was suggestive of a blue nevus.
The MRI of his brain showed a large contrast-enhanced extra-axial mass with solid and cystic components measuring 4 cm x 2 cm x 3 cm in the left cavernous sinus extending through the Meckel’s cave into the posterior fossa (Figure ). A whole-body positron emission tomography-computed tomography (PET-CT) scan confirmed no extracranial disease. He underwent a left temporal craniotomy and zygomatic osteotomy, and we took an interdural middle cranial fossa approach for the radical excision of the tumor.
Perioperatively, we noted the blue nevus on the left side of the forehead in the ophthalmic distribution of the trigeminal nerve. The pigmentation extended into the subcutaneous tissue and galea. The diploe of the temporal bone was also pigmented. The entire temporal dural convexity was pigmented completely black as were the dural root sleeves of the trigeminal nerve and the lateral and medial walls of the cavernous sinus. The tumor was localized in the cavernous sinus and had a well-defined capsule surrounding the divisions of the fifth cranial nerve. It extended into the posterior fossa through Meckel’s cave. The tumor was completely removed via the cavernous sinus through an interdural approach.
The surgical specimen revealed a tumor composed of sheets of moderately large polygonal cells with markedly pleomorphic nuclei with evidence of mitotic activity with foci of necrosis, and occasional cells with intracytoplasmic melanin (Figure ). We also saw small segments of nerve containing ganglion cells with perineural deposits of melanin. The tumor cells showed diffuse positivity for S100 protein and Melan A. Occasional cells were positive for human melanin black (HMB)-45. The ki67/mib-1 was 20% to 25%.
Given the coexistent skin lesion reported as a blue nevus, neurocutaneous melanoma was considered as the provisional diagnosis. The postoperative hyperacute MRI showed no residual tumor. His symptoms gradually resolved after surgery. He received postoperative intensity modulated radiotherapy to the tumor bed (4950 cGy in 22 fractions to the planning target volume with a biologically effective dose [BED] of 60.39 Gy and an equivalent dose in 2 Gy fractions [EQD2] of 50.32). He was closely monitored during the follow-up period.
He presented again about 20 months later with recurrent symptoms of headaches for two months and diplopia and vomiting for two days. On examination, sensations over the V1, V2, and V3 dermatomes were reduced on the left side. There was masseter and temporalis muscle wasting on the left side with impaired blinking in both eyes. His spino-motor system and higher mental functions were found to be normal.
A lobulated heterogenous signal intensity mass lesion was seen in the left CP angle and Meckel’s cave. The mass is predominantly isointense and weighted T1, and T2 images were hypointense (Figure ). Various other similar intensity lesions were seen along the left tentorium suggestive of recurrent meningeal carcinomatosis.
The brain MRI with contrast showed a recurrent mass in the left Meckel’s cave extending into the CP angle compressing the brainstem. Another lesion of a similar nature was seen in the lateral aspect of the cerebellum. There was evidence of thickening and abnormal enhancement of the adjacent pachymeninges and leptomeninges suggesting disease recurrence with leptomeningeal spread. He was further evaluated with a CT of his thorax and abdomen which ruled out extracranial disease.
He underwent a left retromastoid suboccipital re-exploration and subtotal excision of the tumor. A postoperative CT scan of his brain showed no residual disease. However, the patient died five months after the second surgery.
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pmc-6235646-1
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A 28-year-old woman with a history of uterine malignancy, deep vein thrombosis, and hepatitis B, who had been recently admitted as an inpatient for management of renal calculi presented with left knee pain. A magnetic resonance imaging (MRI) scan of the left knee was subsequently ordered by the patient’s clinician. Prior to prescribing an MRI of the left knee, the patient had undergone a recent computerized tomography (CT) scan of the abdomen and pelvis, which had demonstrated numerous partially calcified granulomas in the gluteal subcutaneous tissues bilaterally (Figure ). Additionally, a recent chest CT demonstrated similar findings of prior free-silicone injections within the bilateral breasts and surrounding granulomatous change (Figure ). MRI of the left knee revealed scattered, small circumscribed areas of signal abnormality in the posterior distal thigh, as well as within the subcutaneous tissues and fascia of the popliteal fossa. These round structures were low in signal on proton density, T1, and T2-weighted sequences (Figure ).
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pmc-6235647-1
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A 59-year-old Haitian male with a past medical history of uncontrolled diabetes mellitus was found unresponsive at work. He is a landscaper and was taking his usual lunchtime nap under a tree when his coworkers could not awaken him, prompting them to call emergency services. Upon reaching the patient, paramedics administered 0.5 mg of naloxone intravenously with no effect. Still unarousable, he was transported to the emergency department.
Remaining history and review of systems were limited by the patient’s condition. Physical exam on arrival to the emergency department revealed a stuporous, nonverbal patient who was unresponsive to verbal stimuli. He had minimally reactive, unequal pupils, with the right measuring 4 mm and the left measuring 1 mm. He moved all his extremities in response to painful stimuli. A computed tomography (CT) scan of the head without contrast revealed no acute intracranial pathology. CT perfusion images, CT angiography, and iSchemaView RAPID neuroimaging technology showed no evidence of large vessel occlusion (Figure ). Based on his vague presentation, the paucity of focal findings, and the lack of evidence of ischemia on imaging studies, there was very low suspicion for acute stroke at that time and he was, therefore, not a candidate for tissue plasminogen activator (tPA). Seven hours after his last witnessed normal baseline, diffusion-weighted magnetic resonance imaging (MRI) revealed acute infarcts in the bilateral thalami extending toward the ventral midbrain (Figures -).
As part of the routine stroke work up, an echocardiogram showed evidence of left to right shunting suggestive of a patent foramen ovale, which was closed during his hospitalization. His clinical picture gradually improved over the course of his hospital stay, but he remained with several neurological deficits. By hospital day 22, he was awake and alert, and had recovered speaking and swallowing functions. However, he continued to suffer from recurrent falls, increased impulsivity, and impairments in cognition and memory.
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pmc-6235648-1
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We report a 41-year-old man with history of hyperuricemia and gouty arthritis who presented with progressive dyspnea of three days duration. The patient endorsed multiple painful swelling in his hands and elbow with limitation of motion. He also mentioned new lesions in his ear lobes. The patient is a former smoker with 10 pack year history. He worked as agricultural field laborer and truck driver in El Salvador for six years before immigrating to the United States. Review of system is positive for nocturia.
Examination revealed pale and icteric gentleman with yellowish deposits in both ear lobes (Figure ). Auscultation revealed bi-basilar fine crackles. Also, the patient had joint swelling and deformity associated with hotness and redness in his right elbow, right and left hand, proximal interphalangeal joint, distal interphalangeal joint, and metacarpophalangeal joint (Figure ). Swellings were mildly tender to palpation. Important labs on admission include blood urea nitrogen (BUN) of 65, creatinine of 8.6 and hemoglobin of 6.8. Extensive laboratory and radiologic investigations for causative factors for CKD were negative. He was subsequently started on hemodialysis following worsening renal function. During the course of admission, he was treated for an acute flare of gouty arthritis of his right great toe with renally dosed colchicine. Also, an arterio-venous fistula was secured before discharge.
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pmc-6235655-1
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A 28-year-old man was brought to our emergency department with severe right hip pain with deformity following a high-velocity motor vehicle accident due to the collision of his car with a truck. He was conscious with Glasgow Coma Scale 15 with stable vital parameters. After an initial assessment of the patient, according to the Advanced Trauma Life Support protocol, a secondary survey revealed his right hip in an abducted and externally rotated position with shortening of the right lower limb. On further examination, the femoral head was palpable in the ipsilateral inguinal region. There was no external bleeding wound and no associated distal neurovascular deficit.
The routine imaging investigations and examination ruled out any head, cervical, thoracic or abdominal injury. Plain radiograph of the pelvis with both hips revealed a pubic-type anterior dislocation of the right hip with ipsilateral greater trochanter fracture. A computed tomography (CT) scan of right hip was also ordered to look for any associated acetabular fracture, intra-articular fragment, occult femur neck or intertrochanteric fracture; it ruled out any associated injury and confirmed anterior hip dislocation with ipsilateral greater trochanter fracture (Figure ).
We performed a closed reduction of the dislocation under sedation within two hours of the accident in the emergency department. The patient was positioned supine and the reduction involved the collective effort of four persons; the pelvis was stabilized by one resident, another person pushed the femoral head into the acetabulum by direct palm pressure while the other two gave continuous axial traction in the extended position followed by flexion and internal rotation. A snap sound suggesting relocation of the femoral head followed this reduction maneuver. The post-reduction plain radiograph of the pelvis showed a congruent reduction of the hip joint along with a displaced fracture of the greater trochanter. Subsequently, open reduction and internal fixation were planned for the fracture in the elective theatre the next day. Under C-arm guidance in the left lateral position, the fracture was reduced and two 6.5 mm partially threaded cannulated cancellous screws were inserted through a mini-incision under spinal anaesthesia (Figure ).
The postoperative period was unremarkable and the patient was kept non-weight bearing on the affected limb for two weeks followed by partial-weight bearing over the next two weeks. The patient was allowed full-weight bearing after one month. At the last follow-up of one year, the patient was asymptomatic with a full range of active and passive right hip joint motion. There was no evidence of hip osteoarthritis or osteonecrosis of the femoral head.
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pmc-6236005-1
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The patient was a 54-year-old man who was diagnosed with squamous cell carcinoma in the mandible on the right side. Under general anesthesia, segmental mandibular resection from the right first molar to the left ramus with bilateral radical neck dissection and perimandibular soft tissue resection were carried out. Immediate reconstruction of the soft tissue defect was performed with a forearm flap and a deltopectoral flap. Mandibular reconstruction was not performed primarily (Fig. ). Horizontal distraction osteogenesis of the remaining mandible was performed 5 months later to reduce the bone defect (Fig. ).
The mandible was reconstructed secondarily using a free vascularized fibula flap 1 year after the first operation. The patient was then evaluated for implant therapy, but computed tomography (CT) and 3-D reconstruction images revealed that the height (12 mm) of the fibula was insufficient (Fig. ).
Vertical distraction osteogenesis of the fibula was performed under general anesthesia 1 year after the mandibular reconstruction. An intraoral incision in the buccal vestibule was performed, along with careful subperiosteal dissection to obtain adequate visibility of the underlying bone, taking care to preserve the lingual mucoperiosteal attachment. Two intraoral distraction devices (TRACK 1.5-mm system, KLS Martin L.P.) were adjusted and temporarily fixed by screws as planned before the osteotomy. After removal of the distractors, the osteotomy was performed with a sagittal saw on the vestibular aspect of the fibula. The distraction devices were fixed again at the planned position by screws and temporarily activated to a distance of approximately 5 mm to ensure correct function during distraction. Finally, the osteotomized segment was repositioned at its initial position, and the wound was sutured. After a 7-day latency period, the distraction devices were activated at a rate of 1 mm/day by turning the device twice a day for 13 days. The bone was distracted by approximately 13 mm (Fig. ).
After a 4-month delay for bone consolidation, the distraction devices were removed, and good ossification was observed in the distracted area. The final bone height increase was 11 mm, as observed on CT and further demonstrated by 3-D reconstruction images, and the vertical discrepancy between the reconstructed mandible and the existing mandible was corrected (Fig. ).
After achieving the desired bone height, the vestibular extension was performed using a tissue-engineered oral mucosa (an ex vivo-produced oral mucosa equivalent: EVPOME) []. Autogenous keratinocytes were harvested from a punch biopsy 4 weeks prior to surgery, placed in a serum-free culture system, and seeded onto a human cadaveric dermal equivalent, namely AlloDerm. Clinically, the EVPOME grafts were easy to handle and exhibited excellent compliance with regard to grafting (Fig. ).
Four dental implants were inserted into the distracted fibula, and primary stability was achieved for all implants. Finally, the implant denture was placed on the mandible (Fig. ).
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pmc-6236005-2
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The patient was a 68-year-old female who had lower gingival squamous cell carcinoma in the left side of the mandible. Segmental mandibular resection from the right lateral incisor to the left ramus and resection with a titanium plate were carried out. The mandible was reconstructed secondarily with a free vascularized fibula flap 1 year and 7 months after the first operation (Fig. ). CT and 3-D reconstruction images demonstrated the insufficient height (15 mm) of the fibula for implant therapy (Fig. ).
Vertical distraction osteogenesis of the fibula was carried out 1 year after reconstruction of the mandible. An intraoral incision was made in the buccal vestibule, and careful subperiosteal dissection was performed to obtain adequate visibility of the underlying bone, taking care to preserve the lingual mucoperiosteal attachment. As in case 1, an osteotomy was carried out after provisionally fixing the distraction device (TRACK 1.5-mm system, KLS Martin L.P.) and the device was re-fixed to confirm that it functioned as planned (Fig. ).
After a 6-day latency period, the distraction devices were activated at a rate of 1 mm/day by turning the device twice a day for 15 days. The bone was distracted by approximately 15 mm (Fig. ).
Osteogenesis was good 4 months after the end of vertical distraction, and an implant simulation was performed. The bone extender was removed, and four dental implants were implanted 6 months after the bone distraction. Finally, the implant denture was placed on the mandible (Fig. ).
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pmc-6236112-1
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Patient 1 was 86–90 years old with hypertension, diabetes and previous sigmoid colon cancer, and he visited the ED with a 30 min history of right hemiparesis. His NIHSS score was 17. The MTT map from PCT showed a perfusion delay in the whole left MCA territory with occlusion of the left distal portion of the main stem of the middle cerebral artery (M1) on CT angiography (CTA). The average values of the right and left cerebral rSO2 were 63.29 and 60.64%, respectively. He was treated with endovascular treatment (EVT) and reached the complete recanalization state.
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pmc-6236112-2
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Patient 2 was 61–65 years old with a history of hypertension and diabetes, and he visited the ED with a 50 min history of altered mental status and right hemiparesis. His NIHSS score was 26. The TTP map from PWI showed a perfusion delay in the whole left MCA territory with a multifocal, small-diffusion restricted lesion in the left MCA region on diffusion-weighted imaging (DWI). The average right and left cerebral rSO2 values were 87.15 and 68.25%, respectively. He was treated with combined recanalization therapy, comprising both IVT and EVT.
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pmc-6236112-3
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Patient 4 was 71–75 years old with hypertension, and she visited the ED with a 5 h history of left hemiparesis with dysarthria. Her NIHSS score was 3, and the MTT map from PCT showed a perfusion delay in the right MCA territory with severe stenosis in the superior portion of the right minor branch and moderate stenosis in the right main branch. The average right and left cerebral rSO2 values were 69.01 and 71.28%, respectively. She was treated with EVT.
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pmc-6236112-4
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Patient 5 was 71–75 years old with hypertension and atrial fibrillation, and he visited the ED with a 250 min history of left hemiparesis and dysarthria. His NIHSS score was 5, and the TTP map showed a significant perfusion delay in the right MCA territory with internal border zone area acute infarctions on DWI. The average right and left cerebral rSO2 values were 61.52 and 65.47%, respectively. He was treated with combined recanalization therapy for occlusion of the proximal ICA.
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pmc-6236112-5
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Patient 6 was 76–80 years old with hypertension and diabetes, and she visited the ED with a 125 min history of left hemiparesis. Her NIHSS score was 18, and the TTP map from PWI showed a perfusion delay in the inferior division region of the right MCA and a mild perfusion delay in the other MCA territory. The average right and left cerebral rSO2 values were 60.64 and 63.29%, respectively. She was treated with combined treatment for occlusion of the proximal portion of M1.
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pmc-6236112-6
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Patient 7 was 51–55 years old without underlying disease, and he visited the ED with a 45 min history of left hemiparesis. His NIHSS score was 18, and the MTT map showed a perfusion delay in the whole right MCA territory. The average right and left cerebral rSO2 values were 48.35 and 64.87%, respectively. He was treated with EVT.
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pmc-6236112-7
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Patient 8 was 71–75 years old with hypertension, diabetes and atrial fibrillation, and he visited the ED with a 44 min history of right hemiparesis and stupor mentality. His NIHSS score was 21, and the MTT map from PCT showed a perfusion delay with a large MTT/cerebral blood volume mismatch in the left MCA and ACA territories. The average right and left cerebral rSO2 values were 71.01 and 39.36%, respectively. He was treated with combined recanalization therapy for occlusion of the proximal ICA.
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pmc-6236112-8
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Patient 9 was 71–75 years old with hypertension, and he visited the ED with a 102 min history of right hemiparesis and dysarthria. His NIHSS score was 16, and the TTP map showed a mild perfusion delay in the whole MCA territory with a perfusion defect area at the basal ganglia and corona radiata. The average right and left cerebral rSO2 values were 67.32 and 61.26%, respectively. He was treated with EVT, and the final diagnosis was left MCA infarction with left main branch occlusion.
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pmc-6236123-1
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A young male, 22 years old, presented to our tertiary eye center with complaints of a painles, gradual decrease in vision both eyes, more in the right for 1 year duration. He gave a history of wearing high power glasses (-12.0 DS) OU since early childhood for distance vision.
On presentation, best corrected visual acuity (BCVA) was hand movements close to face (HMCF) with inaccurate projection of rays (three quadrants) OD and 6/24 (0.6 logMAR) projection of rays accurate OS. Ocular examination revealed a port-wine stain on the right upper lid along with bluish-black scleral pigmentation and dilated prominent episcleral vessels (episcleral vascular malformations i.e., EVM) in both eyes () with a clear cornea, deep anterior chamber, homogenously dense iris pigmentation and a clear lens. Posterior segment examination revealed both optic nerves to be average in size; OD showed a 0.9 cupping with up to 270o neuro-retinal rim loss; OS showed 0.8 cupping with a bipolar notch. There was pigmentation within the inferotemporal optic disc margin bilaterally, without any associated choroidal or retinal pigmentation (). The IOP noted was 50 mm Hg OD and 44 mm Hg OS and required systemic hyper-osmotic agents for control. Gonioscopy revealed wide angle recess with an anterior or high insertion of iris reaching up to the anterior trabecular meshwork at places, along with homogeneously dense trabecular pigmentation and concavity of iris configuration (). The visual fields were not possible in the right eye due to poor vision while advanced field loss, i.e., incomplete double arcuate scotoma with the involvement of fixation was seen in the left eye ().
On systemic examination, bilateral port-wine stain could be noted on the face, over the cheek, upper jaw and chin, more on the left side along with brownish-black pigmentation over temples and forehead extending onto cheek and medial aspect of lower lid bilaterally and also upper jaw on the left side (). Similar pigmentation was seen on the hard palate (Palatal Melanocytosis i.e., PM) centrally along with palatal vascular malformation (PVM) on the left side (). No other port-wine nevi were present on any other body parts. The facial and ocular pigmentation (ODM) had been present since early childhood. There was no history of seizures, hemiparesis or mental retardation. Bilateral varicose veins were noted over the last ten years requiring surgical intervention one year prior (). Neuroimaging by MRI could not document cerebral calcification or atrophy. Family history for similar disposition was negative, thus pointing to a sporadic inheritance.
Due to advanced glaucomatous damage in both eyes and requirement of systemic anti-glaucoma medications for IOP control, the patient was subjected to trabeculec-tomy with anti-fibrotics and releasable sutures in both eyes sequentially at 2 weeks interval. Intraoperatively, care was taken to stabilize the IOP by systemic hyperosmotic agents and controlled paracentesis was done to maintain a stable anterior chamber and help reduce the risk of suprachoroidal hemorrhage. The surgery was uneventful, without the need for draining sclerotomies, but a mild hyphaema occurred at the very end, due to episcleral vessel bleed while suturing the scleral flap. Post-operative course was uneventful, and complications like hypotony, shallow chamber, fresh bleeds or high IOP spikes were not noted. The releasable sutures were removed at two weeks post-operatively. Trabecular meshwork was sent for his-topathology and noted to have increased melanocytes. By 6 weeks’ post-operative, his vision improved to finger counting 1 meter with -14DS OD and 6/18 with -11DS OS; which has remained stable over 3 years follow-up. The IOP is maintained between 9 to 13 mm Hg without any medications.
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pmc-6236524-1
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A 10-year-old girl visited our affiliated hospital, complaining of pudendal deformity. The patient was born at 39 weeks of gestation by normal delivery as the second child, and the birth weight was 3,144 g. There was no disorder in the course of pregnancy in her mother. Intake of androgenic medicine or the disorders of ovaries and uterus was not observed by a periodic medical examination. No pudendal deformity was clearly observed at birth, but lateral asymmetry of the pudendal region was noticed at about 4 years old. A child care worker pointed out that she pressed her heel to the crotch while sitting on her folded legs. After 5 years old, her mother confirmed that her clitoral hood clearly hypertrophied. After entering primary school, a teacher in charge pointed out that she pressed her crotch to a chair or bar, and the mother told her to stop it, but she repeated this behavior every day. When she strongly wanted to be absent from an overnight school trip at 9 years old, the mother brought her to the Pediatric Department. Various tests were performed suspecting disorders of sex development (DSD). On the first visit, the height was 132 cm and the body weight was 26 kg. The clitoral hood was enlarged. The appearance was similar to the vulva in children with congenital adrenal hyperplasia, and the clitoris size was 8 x 5 mm. Labial fusion or adhesion was not detected, and the urinary tract and vagina were open at the normal positions. No masculinization, such as acne and polytrichosis, was noted (). Intake of androgenic medicine or the disorders of prepuce was not observed. In the blood test, the sex chromosome was 46, XX. The blood count, blood chemistry, and hormonal test were normal (). On abdominal ultrasonography, the uterus and ovaries were present. Abdominal CT and MRI examinations showed no tumorous lesion.
Based on the above examination and test findings, DSD was considered negative. The patient was diagnosed with clitoral hood enlargement and referred to our department to undergo clitoral hood reduction. For surgery, a longitudinal incision was designed for the dorsal side in order to resect the clitoral hood by cut and try. In the clitoral region, the clitoral hood and corpus cavernosum were dissected through an inverted V-shape incision. The volume of the exposed corpus cavernosum clitoridis was reduced while conserving the neurovascular bundle, following the Marberger method []. The clitoral hood was resected into a triangle shape and used for labial formation. On histopathological examination, lymphedema and venous tasis in a grade consistent with the influence of foreskin excision were observed. No abnormality was noted in the corpus cavernosum. Her postoperative course was uneventful. As of 10 months after surgery, favorable improvement of the appearance was noted ().
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pmc-6236899-1
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A 54 year-old female presented to the outpatient clinic with weakness and lower extremities paresis, nausea and three times vomiting, after receiving clindamycin, dicloxaciline, and oral acyclovir 400 mg each 8 h were prescribed to treat a dental abscess. Relevant past medical history included allergy to penicillin and smoking for 20 years. Physical examination disclosed good hydration status, decrease in the patellar osteotendinous reflexes (++ / ++++). Initial laboratory data showed a serum creatinine level of 2.1 mg/dL; blood urea nitrogen 86.4 mg/dL; serum potassium 2.1 mmol/L, sodium 134 mmol/L, phosphorus 1.7 mg/dL, and magnesium 2.15 mg/dL. (Table ).
The patient was advised to stop the medications and to start with oral potassium supplement. She noticed improvement of the weakness; however, due to persistent hypokalemia, the patient was admitted to the hospital for further evaluation; new blood test showed a serum creatinine level of 1.7 mg/dL and blood urea nitrogen 76.3 mg/dL; serum potassium 2.5 mmol/L, sodium 139 mmol/L, phosphorus 2 mg/dL, and magnesium 1.88 mg/dL; urine sediment was unremarkable; 24 h urine potassium was 49 mEq/day, sodium 86 mEq/day, and calcium 89 mg/day. A renal ultrasound showed a normal-size kidney, without hydronephrosis. Kidney biopsy was performed.
Two fragments of kidney tissue were obtained (Fig. ), By stereoscopic evaluation 11 glomeruli were identified. By light microscopy 9 glomeruli were observed; three were globally sclerosed and the remaining glomeruli were normal. Tubular atrophy was seen in 15–20% of the tubules; the remaining tubules showed vacuolated, granular cytoplasm, focal sloughing of the epithelium, and regenerative changes of the brush edge of tubular cells and hyaline casts (Fig. ). By Masson’s trichrome stain (Fig. and ), the interstitium exhibited 0–15% fibrosis, with inflammatory infiltrate by lymphocytes, plasma cells, and few eosinophils, that penetrated the tubular epithelium. Arterioles and medium-caliber arteries were permeable without vasculitis or thrombi. By Jones (Fig. ) tubule-interstitial nephritis infiltrated with abundant inflammatory cells were observed. Immunofluorescence (Fig. ) was negative for IgM, IgG, IgA, C3c, C1q, kappa, lambda, fibrinogen, and albumin. Histological findings were consistent with acute tubular necrosis and acute tubulointerstitial nephritis.
Due to persistent hypokalemia oral and intravenous potassium replacement was started with good tolerability and no adverse events, symptoms improved; the patient was discharged 4 days later with a serum potassium of 3.1 mEq/L; oral replacement of potassium was continued until serum potassium levels were > 3.5 meq/L, after few days later she restart her normal life.
Hypokalemia and acute kidney injury due to oral acyclovir.
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pmc-6236921-1
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A 22-year-old male presented at the hospital because of a rash, joint pain for four months, and breathlessness for one month. Five months before admission, he had tattooed a butterfly on his right chest with blue and red ink (). Then, four months before admission, erythema appeared on multiple parts of the skin, including the face, the extensor surface of the bilateral elbow, the metacarpophalangeal joints (MCP2–4), the neck, the chest, and the right side of the back (Figures and ). However, there was no muscle weakness. Gradually, he began to develop shortness of breath after physical activity. A computed tomography (CT) scan of the chest indicated ILD (). Physical examination showed typical Gottron rash. C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), rheumatoid factor (RF), electrolytes, glucose, hepatic/renal function, and hepatitis (A, B, and C) were all normal. Laboratory findings of antinuclear antibodies (ANA), extractable nuclear antigens (ENA), anti-centromere antibodies (ACA), complement (C3, C4, and CH50), immunoglobulin (IgM AG), antineutrophil cytoplasmic antibodies (ANCA), antinucleosome, cyclic citrullinated peptide (CCP) antibody, and glycoprotein I (GPI) were all within the normal range. He had normal creatine kinase levels (CK 32 U/L) and significantly increased levels of ferritin (1016.9 ng/ml). The above findings represent the diagnosis of CADM and ILD. He was treated with glucocorticoid and cyclosporin A (CsA). According to his history, we deduced that the CADM was caused by a tattoo in his right chest. So, the tattoo was surgically resected, and dermatopathologic analysis of the blue and red tattoo was performed with hematoxylin and eosin (HE) stain. There was no hyperplasia of the epidermis. Pigmentation associated with a small number of inflammatory cells and hyalinization of collagen fibers was detected in the superficial dermis. But no significant difference of lymphocytic infiltration was detected between the blue and the red part of the tattoo (Figures –). After treatment, the rash and the ILD gradually improved (), and the patient was discharged from the hospital.
During the follow-up, he was admitted to our hospital again for shortness of breath after even minor activities a week after he was discharged. A CT scan indicated advanced ILD (). His blood routine test, electrolyte, CRP, ESR, liver function, and serum creatinine levels were within the normal range. T-SPOT and (1,3)-β-D glucan tests were also normal. The ferritin level was 888.6 ng/ml. He was treated with CsA and methylprednisolone. Amphotericin B, norvancomycin, cefoperazone-sulbactam, and SMZ were used to treat potential infections. Cyclophosphamide (CTX 0.4 g) and gamma globulin (10 g) were also intravenously injected. However, both the cytomegalovirus (CMV) and antibodies (IgG/IgM) were positive. So, ganciclovir was used to replace the amphotericin B and cefoperazone-sulbactam. Ten days later, the patient had a fever and felt breathless. Chest X-rays indicated diffused lesions in the bilateral lung. He was given BiPAP to assist ventilation. Imipenem-cilastatin, teicoplanin, and levofloxacin were used to control infection. Intubation was also conducted, as the oxygen saturation was continually lower than 80%. A lab test showed that white blood cell (WBC: 20.0 × 109/L), ESR (53 mm/h), and CRP (52.9 mg/l) levels were obviously elevated. Blood gas indicated a low level of oxygen pressure (58.9 mmHg). Although the patient had a definite inducement, his respiratory failure gradually aggravated. Finally, he was dead of respiratory failure.
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pmc-6236949-1
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A 64 year-old caucasian male presented with a chief complaint of nausea and vomiting. These episodes occurred three-to-four times per day for the past 3 days and were non-bilious, non-bloody, mostly foodstuff. There were no precipitating factors or associated symptoms including abdominal pain or diarrhea. He did not complain of any recent pulmonary symptoms such as cough, hemoptysis, dyspnea, or chest pain, and denied any fevers, night sweats, or weight loss. He had no past medical history except for hypercholesterolemia controlled with atorvastatin. His only family history included Hodgkin’s lymphoma. Smoking history revealed 20 pack years and quit 3 months prior to his visit. Vital signs upon presentation were unremarkable. Physical examination revealed mild right upper lung field end-expiratory wheezing, no clubbing of his digits, no jugular venous distention, no lower extremity edema, was euvolemic, and had unremarkable abdominal findings.
An anterior-posterior chest plain film (Fig. ) was performed in the emergency department, and read by the radiologist as having no evidence of acute cardiopulmonary disease. Laboratories drawn on admission revealed hyponatremia in the context of a low serum osmolality and a high urine osmolality (Table ). Given these laboratory findings, SIADH ranked high in our differential diagnoses.
Treatment with fluid restriction was initiated and sodium levels gradually improved (Table ). The patient’s nausea and vomiting had resolved as his sodium levels improved, which later was attributed to his hyponatremia from SIADH. Potential etiologies for SIADH (i.e. infectious, cerebral, medications, endocrinopathies) were further investigated and were unremarkable. Due to the patient’s significant smoking history, unilateral end-expiratory wheeze, initial poor quality chest imaging, and high-index of suspicion, a CT chest was ordered. It revealed the presence of a conglomeration of nodules and opacities measuring 3.0 × 1.9 cm in the inferior segment of the right upper lobe of the lung with ipsilateral mediastinal lymphadenopathy. Subsequent CT-guided percutaneous biopsy was performed. Biopsy specimen stained positive for CK, CD56, NSE, BcL2, Synaptophysin, PAX-5, and TTF-1, but negative for Chromogranin, CD57, and NFP; consistent with small cell carcinoma. Further metastatic workup including CT abdomen and pelvis along with brain MRI was performed, which showed no evidence of metastasis.
He was subsequently discharged with an appointment with an outside oncologist near his home as he lived far from our institution. A follow-up telephone conversation occurred 4 weeks from discharge. The patient’s oncologist had ordered a whole-body PET scan within 1 week of hospital discharge and it revealed uptake in one ipsilateral mediastinal lymph node. He was classified as having limited stage small cell lung cancer (LS-SCLC) and was undergoing chemotherapy with plans for subsequent prophylactic cranial irradiation. At 4 months follow-up, he had completed three chemotherapy sessions and prophylactic cranial irradiation. His oncologist had deemed him to be in remission and undergoing continued surveillance.
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pmc-6236950-1
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A 22-year-old male amateur basketball player with no relevant medical history was admitted to our hospital due to continuous severe lower back pain with radiating nerve pain and numbness from the hip to the posterior part of the left leg. Physical examination revealed paravertebral muscle spasm, diminished sensation at the lateral aspect of the sole of the left foot, diminished strength in plantar flexion on the left side, a weakened Achilles tendon reflex on the left side, and a positive straight-leg raise test (30 degrees) on the left side. Magnetic resonance imaging of the lower spine showed a herniated nucleus pulposus at the left L5-S1 level, and so the patient was diagnosed with left lumbar disc herniation at L5-S1.
Preoperative evaluation was normal. The patient opted to undergo surgical treatment with PEID after failure of conservative treatment. Tracheal intubation was facilitated via the administration of atracurium and penehyclidine. General anesthesia was induced with propofol and fentanyl, and was maintained with sevoflurane, fentanyl, and atracurium. The PEID was successfully completed within 40 min, with complete removal of the herniated disc, annuloplasty of the annulus fibrosus, and thorough decompression of the S1 nerve root. Intraoperatively, the patient was infused with 1,100 mL of Ringer’s lactate solution. Throughout the entire procedure, the urine volume was 400 mL, and the blood loss was less than 20 mL.
Extubation was performed when the patient was conscious, spontaneously breathing, and performing purposeful movements. The patient then suddenly began to respire forcefully. The heart rate was 130 beats/min, blood pressure was 155/90 mmHg, respiratory rate was 35 breaths/min, and SpO2 had decreased from 98 to 65%, followed by the production of 5 ml of pink frothy sputum. Chest auscultation performed by the anesthetist revealed tachycardia and dispersed moist rales bilaterally. Arterial blood gas analysis showed that the pH was 7.34, PaO2 was 71 mmHg, and PaCO2 was 40 mmHg. Electrocardiography indicated sinus tachycardia. NPPE was diagnosed. The airway was kept unobstructed, and oxygen was delivered at 5 L/min via mask ventilation. The infusion volume was limited. Dexamethasone was administered to relieve spasm, and 20 mg of furosemide was administered intravenously to treat pulmonary edema. The SpO2 improved and was maintained above 93%.
After stabilization with supportive treatment for about 60 min in the operating room, the patient was transferred to the orthopedic ward. Subsequent laboratory testing indicated an increased white cell count of 12.5 × 109/L, an elevated neutrophil percentage of 84%, and a hematocrit decrease from 45.3 to 40.7%. The D-dimer level was 1.0 ng/mL, while the other main laboratory parameters were within normal limits. Repeat arterial blood gas analysis indicated that the pH was 7.37, PaO2 was 95 mmHg, and PaCO2 was 48 mmHg with oxygenation via nasal catheterization. Compared with the normal chest radiograph preoperatively (Fig. ), the emergency chest radiograph showed bilateral infiltrates with opacities and nodules (Fig. ). Two hours later, chest computed tomography (CT) of a pulmonary window showed increased vascular diameter, a mosaic pattern of attenuation, and bilateral pleural effusion (Fig. ).
The patient was afebrile, and cefazolin was administered prophylactically. The oxygen requirement gradually decreased to room air, with the hematocrit remaining stable, and the vital signs remaining stable for the following 24 h. A chest radiograph obtained in the morning on postoperative day 1 still showed bilateral infiltrates with opacities and nodules; however, the severity of these was obviously decreased compared with the radiograph taken the previous evening (Fig. ). A chest radiograph taken in the afternoon on postoperative day 1 showed that the infiltrates had further decreased. In the evening on postoperative day 1, CT showed a marked decrease in bilateral pleural effusion (Fig. ). The vital signs were stable, without tachycardia, tachypnea, and/or hemoptysis. At 3 days postoperatively, radiography showed nearly complete resolution of the lung infiltrate (Fig. , ) and CT (Fig. ). The patient was discharged 3 days after spinal surgery, at which time he reported that he was satisfied with the whole therapeutic process, and had marked improvement of the symptoms in the back and left leg.
At 6 weeks postoperatively, the patient was examined in the outpatient department. The pulse oxygenation was 100% on room air, and there were no abnormal findings on chest auscultation, radiography (Fig. ), and CT (Fig. ).
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pmc-6236957-1
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A 56-year-old nonsmoking woman consulted for a painful mass in her left thigh. Her past medical history was only significant for untreated autoimmune hepatitis. MRI of her left thigh showed a posterolateral muscular mass measuring five centimeters, with both necrotic and enhanced portions (Fig. a). Histologic analysis performed on biopsy samples showed evidence of malignant pleomorphic proliferation suggesting a diagnosis of high-grade fibrosarcoma. However, the results were not totally conclusive because of pan-cytokeratin AE1/AE3 expression that could also be found in sarcomatoid carcinomas. The diagnosis of pleomorphic fibrosarcoma was finally maintained due to the lack of epithelial marker CK7, CK5/6 and p63. (French Federation of Cancer Centers Sarcoma Group grading system [FNCLCC] = Differentiation: 3; Necrosis: 2; mitosis: 10 High-Power Field: 35; Mitotic Index: 3; Grade: 3).
A thoracoabdominal CT scan was performed for tumor staging and found a 35-mm saccular aneurysm of the descending thoracic aorta (Fig. b–d). The patient, despite her age, had no cardiovascular risk factors. A PET-CT performed 1 month later showed a suspicious contralateral limb metastasis and abnormal aortic FDG uptake around the aneurysm, which could be attributed to an infectious or tumorous process (Fig. e). Follow-up CT scans showed quick growth of the aneurysm from 35 to 49 mm. The patient was then transferred for endovascular aortic repair with an endoprosthesis (Fig. f) measuring 28 × 164 mm (Relay NBS® Bolton Medical). The presence of an atypical epigastric artery angiogram led to an artery biopsy during the procedure, which found no evidence of dysplasia. Despite negative blood samples taken near the aneurysm, the possibility of an infectious location was discussed due to the patient’s poor dental condition. Postoperative probabilistic antibiotic treatment was started with oxacillin and ofloxacin, and then amoxicillin.
Surgical resection of the primary tumor was rejected due to histological confirmation of a right gluteal metastasis (contralateral). Initially, the patient received conventional chemotherapy by doxorubicin and ifosfamide, but the treatment was quickly switched to cisplatin and paclitaxel due to tumor progression. The patient was then included in a clinical trial (NCT01308034 Study of Continuous Dosing of Sunitinib in Non GIST Sarcomas with Concomitant Radiotherapy) and treatment by sunitinib was introduced with concomitant radiotherapy. Due to adverse effects that led to a deep thrombocytopenia (50 G/L), sunitinib was stopped.
Follow up CT scans (Fig. a–c) showed the appearance of a suspicious lesion near the excluded aneurysmal cavity with contrast-enhanced portions (Fig. a, b). Aortic MRI and contrast-enhanced ultrasound confirmed the presence of tumor tissue instead of thrombotic material within the aortic aneurysm sac (Fig. c–f). A transparietal biopsy of the large mass was performed using ultrasound guidance by a left posterior paravertebral approach, and histological examination found pleomorphic spindle cells with pan cytokeratin and smooth muscle actin positivity which confirmed the diagnosis of sarcoma metastasis (FNCLCC grade 3). Palliative management was decided. The patient was included in another clinical trial (NCT02406781-PEMBROSARC) and received four injections of pembrolizumab during a 3-month period, associated with cyclophosphamide. The patient died due to mediastinal tumor progression 17 months after initial sarcoma diagnosis.
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pmc-6236987-1
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A 55-year-old man presented with chronic testicular pain. An ultrasonography of the abdomen detected left renal tumor. The patient had a history of hypertension and left renal urolithiasis. CT showed a heterogeneous left upper pole renal tumor (5.3 cm in diameter). A laparoscopic radical nephrectomy was performed in May 2008. Left adrenalectomy and lymph node dissection were not performed because the CT scan showed no adrenal gland invasion or lymphadenopathy. The histological evaluation of the tissue revealed a clear cell renal cell carcinoma and negative surgical margins (pathological stage, T2N0M0). Three years after nephrectomy, following a cerebrovascular accident, the Eastern Cooperative Oncology Group score changed from 0 to 2. No tumor recurrence (CT scan was performed every 6 months) was found until 51 months later. A CT scan detected two nodules in the renal fossa (1.8 and 0.9 cm, respectively). Retroperitoneal exploration confirmed recurrent clear cell carcinoma with microscopically positive surgical margins. Lymph node dissection was not performed because of severe adhesion around the aorta. Lymph nodes that could be detected by palpation were not identified during the surgery. Four months after excision, an abdominal CT showed a nodule (1.6 cm) over the right adrenal gland. At that time, tumor target therapy was not covered by the national health insurance in Taiwan. Therefore, because of economic reasons, the patient could not afford the treatment until 2013. A repeat CT evaluation confirmed the disease progression of the adrenal metastasis (2.1 cm). The patient was treated with sunitinib (37.5 mg/d) for 4 weeks, but the treatment was discontinued because of gastrointestinal side effects and fatigue. After 3 months, a CT scan showed the progression of the adrenal metastasis (3.8 cm) and no lower lung lesion. A chest X-ray revealed the absence of lung metastasis. The patient refused to undergo hormonal survey, biopsy, and adrenalectomy. Eleven months after sunitinib treatment, a CT scan showed an obvious growth of the adrenal metastasis (5.7 cm) (Fig. a), whereas 16 months after the treatment, a regression of the metastasis (3.4 cm) was observed (Fig. b). Twenty-two months after sunitinib treatment, a CT scan demonstrated a gradual reduction in the size of the adrenal metastasis (1.8 cm) (Fig. c). The patient was still alive and followed up at the outpatient department 44 months after the discontinuation of sunitinib treatment.
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pmc-6237518-1
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A 56-year-old female presented with a complaint of left flank pain for two weeks. Her past medical history was unremarkable. A left pelvic calcification was observed on abdominal X-ray (). Ultrasonographic examination () revealed a grade II left ureterohydronephrosis and heterogeneous cystic mass in left ovary, and CT was performed to confirm diagnosis, showing a well-defined 5.4 × 4.3 × 4.5 cm left adnexal lesion () with fat and calcification, compressing distal ureter and gonadal vein that were dilated () as a consequence of the compression by ovarian mass. Tumor markers (CA125, CEA and CA19-9) were with in normal range. Under a clinical diagnosis of ovarian germ cell tumor, laparoscopic salpingo-ooforectomy was performed. Histopathological examination of the specimen () revealed mature hair follicles, sebaceous glands, fat cells and mature nervous tissue, typical features of a mature cystic teratoma (MCT).
Ovarian MCT is a cystic or solid tumor (composed of mature, adult type tissues) which accounts for 10-20% of all ovarian tumors (). Malignant transformation occurs in less than 2% (). Ovaries are close to pelvic urological organs, such as ureter and bladder, so ovarian masses can often impinge upon these adjacent organs and develop symptoms like pain, urinary and gastrointestinal complaints (). Ovarian cancer is described as the most common cause of malignant extrinsic ureteral obstruction (16.6%) (), but the exact prevalence of ureteral involvement by ovarian MCT is still unknown. The differential diagnosis of calcifications in abdominal plain films of the female pelvis include: vascular calcifications (atherosclerosis, calcified aneurysms, phleboliths), those originating from the urinary tract (ureterolithiasis and vesical lithiasis), inflammatory masses (epiploic calcifications, dropped gallstones, foreign bodies) and nodal calcifications ().
Early diagnosis and treatment in terms of a conservative surgical approach is recommended. Ovarian MCT should be considered in the differential diagnosis of distal ureteric obstruction causing proximal hydroureteronephrosis in young female patients ().
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pmc-6237544-1
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A 24-year-old pregnant woman presented to a local private clinic with severe intermittent headache. She was at the 17th week of her first single fetus pregnancy. On physical examination, the patient had systolic blood pressure at 220-240mmHg and diastolic blood pressure at 140mmHg. No other abnormalities were noted. With diagnosis of pregnancy-induced hypertension (PIH), antihypertensive treatment was begun and patient was referred to an obstetrics clinic. Gynecologist refused the diagnosis of PIH, because, PIH develops after 20 weeks of gestation. Past medical history revealed a history of palpitation and sweating for about 1 year and she has not undergone any medical workup. During pregnancy, her blood pressure ranged from 125/75mmHg to 145/85mmHg. Despite the maximal dose of antihypertensive treatment, BP persisted uncontrolled. Abdominal ultrasonography showed a mass measuring 31×33mm medial to left renal hilum. The results of laboratory studies, including blood cells count, blood chemistry, urine analysis, urinary albumin and blood electrolytes, were within normal limits. Hormonal examination showed markedly elevated 24-hour urinary excretion of metanephrines and normetanephrines. The other hormonal assessment including adrenocorticotropic hormone, cortisol, aldosterone and plasma renin activities were in normal range. Magnetic Resonance Imaging (MRI) of abdomen showed a round soft tissue mass measuring 3×3.5cm medial to left renal hilum, anterior to renal artery and vein (). This tumor was compatible with extra adrenal pheochromocytoma (paraganglioma).
Alpha-adrenergic blockade with phenoxybenzamine was performed for 10 days and blood pressure was maintained under 140/90mmHg. At 19 weeks of gestation, she underwent laparoscopic tumor removal. Laparoscopy was done by the transperitoneal approach in left flank position, as it best exposes the tumor and renal vessels. We used the Hasson technique to create pneumoperitoneum and the operation was done by four working trocars. It was necessary to mobilize the colon and tail of the pancreas. The tumor was located very adjacent to the major blood vessels of the left kidney (). The tumor was excised effectively without any renal vascular damage. The operation was uneventful and the patient blood pressure was controlled without medications. She was discharged after 5 days with a blood pressure of 140/75mmHg and a heart rate of 86 beats per minute. Microscopic histopathology revealed extra adrenal pheochromocytoma (paraganglioma). The patient had a normal vaginal delivery of a healthy baby at 39 weeks of gestation.
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pmc-6237918-1
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A 55-year-old man with a history of progressing skin lesions over the past 8 months visited our department for the first time in spring 2011. The clinical examination revealed multiple erythematous papules and plaques with crusts on his back, chest, face, and scalp (about 40% of body surface area was involved) with no mucosal involvement (Figure ). The patient presented no other symptoms and had no chronic diseases or allergies. His blood tests revealed a highly elevated Dsg1 antibody level (130 U/ml; normal range < 20 U/ml) and a slightly elevated γ-glutamyltransferase level. Differential blood count, liver enzymes, creatinine, and Dsg3 antibody level were within the normal range. Histological examination of the patient's skin biopsy revealed an inflammatory infiltrate, eosinophilic spongiosis, and superficial epidermal blister formation.
Based on the findings, pemphigus foliaceus was diagnosed and a treatment with prednisolone (10 mg/day) and azathioprine (100 mg/day) was started. Topical therapy with clobetasol propionate and chlorhexidine was also initiated. Furthermore, methylprednisolone infusions (750 mg) were administered once a month for 3 months. This treatment did not result in complete remission; thus, methylprednisolone was replaced with dexamethasone (300 mg) and cyclophosphamide infusions (500 mg) once a month. Azathioprine had to be discontinued due to increasing liver enzymes. The treatment with cyclophosphamide and glucocorticoids was discontinued after 5 months without achieving remission. Hence, we next treated the patient with rituximab. Therefore, two rituximab infusions (1 g each) were administered 2 weeks apart leading to a near-complete b-cell depletion in peripheral blood, a decrease in Dsg1 antibody levels (below the detection range), and an almost complete remission of the skin lesions within the next year (Figure ). Consecutively, therapy with prednisolone (10 mg/day) and topical mometasone furoate was continued and in the following 2 years, the prednisolone dose was reduced to 5 mg/day. The patient remained in remission for 7 years with this therapy (with Dsg1 antibody levels continuously within the normal range). However, in autumn 2017, skin lesions reappeared, which was accompanied by an increase in the Dsg1 antibody levels (75 U/ml). The prednisolone dosage was increased (temporarily up to 60 mg/day), but it was not sufficient to control the disease. Therefore, rituximab infusions (2 × 1 g within 14 days) were readministered, which led to slow continuous healing of the skin lesions.
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pmc-6238255-1
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During November 2016 an 11-year-old girl affected by MPS type VI had been referred to our system for medical examination. She had full and intense clouding in both eyes with a corrected visus of only 0.1 in the left eye and 0.2 in the right eye. She was therefore subjected to preliminary examinations (slit lamp, tonometry, and fundus), the execution of which proved very challenging; nevertheless, she did not present any major anomaly.
In December 2016 she underwent a penetrating keratoplasty procedure on the left eye. During the following check-ups the suture was fine, the graft was transparent, the intraocular pressure was normal, and the visus kept improving up to the value of 0.4–0.5 (corrected) measured during her latest check-up in March 2017. No inflammatory signs were detected and the patient was very satisfied with her new visual capability.
A corneal topography had been executed during every examination and showed, during the last check-up, a regular astigmatism of 3 D, and a biomicroscopy of the graft showed a cellular density of 2250 cells/mm2 and a graft pachymetry of 404 μm.
No subjective disorder has been reported by the extremely compliant little girl or by her parents.
The patient is following the above-mentioned postsurgical therapy protocol aimed at preventing inflammation and rejection.
Given the good results already achieved and the high possibility of further improvement, especially when the suture will be fully settled allowing an optimal correction, a transplant will also be considered in the contralateral eye to regain a full binocular view and prevent, as far as possible, any phenomenon of amblyopia (lazy eye) (Fig. ).
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pmc-6238297-1
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A 6-year-old girl with MPS IVA who had previously undergone CVJ decompression at another institution for severe canal stenosis and mild myelopathy, with removal of the posterior arch of C1 and of the thickened atlanto-occipital membrane and ligamentum flavum. After a backwards fall from a child’s chair, she developed acute quadriplegia with respiratory failure (Ranawat IIIB) and was admitted to our neurological intensive care unit. MRI showed an impressive alteration of spinal cord signal at C0–C1 (Fig. ). This patient was initially stabilized with an external halo orthosis and submitted to inpatient rehabilitation for some weeks afterwards. After an initial neurological improvement and cardiorespiratory stability, she underwent internal stabilization with C2 pars screws (Fig. ) anchored to an occipito-cervical U-loop and occipito-C2 calvarial bone graft. At the 4-year follow-up examination she was able to walk with crutches (Ranawat IIIA). Radiological follow-up examinations revealed wide canal decompression and a stable construct (Fig. ). This case supports the evidence that stabilization should be always recommended and that the placement of an external orthosis may still represent a valid treatment option in selected cases (e.g., impossibility to perform surgical intervention for respiratory instability).
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pmc-6238297-2
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A 6-year-old male with MPS VI. After a minor fall he experienced a transient tetraparesis with quick recovery of ambulation. In the following months he suffered from recurrent urinary tract infections. Cervical MRI documented severe stenosis and cord compression at the CVJ with spinal cord signal alterations. Physical examination evidenced pyramidal signs and a urodynamic study was diagnostic for neurological bladder. A posterior cervical decompression and stabilization with C2 pars screws anchored to an occipito-cervical U-loop and calvarial bone graft was then performed. During the follow up, there was a slow recovery of bladder function and normal daily activities. Radiological follow-up examination revealed good canal decompression, stable construct, and steady neurological conditions.
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pmc-6238297-3
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A 2-year-old boy with MPS IVA. During routine neuroradiological workup, severe canal stenosis > 50% at the CVJ was observed without signs of myelopathy. Due to the young age of the patient, incomplete development of bony structures at the CVJ, and increased risks of general anesthesia, surgery was schedule at 3 years of age. One year later, follow-up dynamic MRI showed increasing spinal cord compression in flexion (Fig. ), although the absence of myelopathy persisted. A preventive CVJ decompression and internal fixation with C2 laminar screws (Fig. ) anchored to an occipito-cervical loop augmented with calvarial bone was then performed. Follow-up showed a stable construct (Fig. ), without any relevant complication.
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pmc-6238309-1
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A 54-year-old Caucasian man sought care because of a 2 weeks history of jaundice and intermittent fever (up to 39 °C), not responsive to antibiotics and antipyretics. His past medical records included arterial hyperthension and a left vertebral artery dissection. Upon admission, he was pyretic, jaundiced, tachypneic and lypotimic. No cutaneous lesions were present. Neurological examination was normal. Laboratory tests showed low blood oxygen concentration (pO2 62 mmHg, pCO2 22 mmHg, HCO3 18.8 mmol/L, pH 7.55), anemia (Hb 10.2 g/dL), leukocytopenia (3.100/mcL) and thrombocytopenia (62.000/mcL). Atypical circulating lymphocytes were absent. Increased levels of transaminases (ALT 1374 u/L; AST 654 u/L), gamma-GT (802 u/L) and lactate dehydrogenase (LDH 2998 u/L) were present. Serum microbiological tests were negative. Computerized tomography (CT) scan revealed hepato-splenomegaly and diffuse ground-glass opacities in both lungs without interlobular septal thickening. No lesion was detected in the upper aerodigestive tract. Despite oxygen therapy, the clinical conditions rapidly deteriorated leading to death 3 days after admission. A severe, generalized sepsis was suspected. A total-body autopsy was performed.
Gross examination revealed pericardial, pleural and peritoneal effusions. The lungs were heavier than normal (right lung 910 g; left lung 930 g) with multiple foci of consolidation. The spleen was enlarged (610 g) as well as the liver (1920 g), without focal lesions. No lesions were found in the skin, oral cavity or oropharynx. Polymerase chain reaction (PCR) detected about 2 millions copies of EBV DNA on pleural (Fig. ) effusion and lung tissue.
Histology revealed atypical lymphoid cells filling and expanding the lumina of small and medium blood vessels (Fig. ) in virtually any organs (heart, lung, kidney, spleen, liver, brain). A sinusoidal involvement occurred in bone marrow, with about 10% of neoplastic infiltrate. Features of hemophagocytosis (Fig. ) were also evident in bone marrow. The lymphoid cells, strikingly confined to the blood vessels lumina, were large-sized with hyperchromatic nuclei and expressed at immunohistochemistry: CD3 (Fig. ), CD2, perforin, CD56 (Fig. ), granzyme B (Fig. ), showing a T and cytotoxic phenotype. CD20, CD79alfa, PAX5, CD4, CD8, CD5, ALK1, CD16 were all negative. Either external or internal positive controls were used to validate the assay for each immunohistochemical staining. The proliferative index (Ki67) was high (approximately of 80%). EBV encoded RNA in situ hybridization (EBER) was diffusely positive (Fig. ). Polymerase chain reaction identified a clonal T-cell receptor gamma gene rearrangement (Fig. ). The final diagnosis was EBV positive intravascular NK/T-cell lymphoma with multisystem involvement.
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pmc-6238337-1
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A 75-year-old white man presented 1 day after uncomplicated phacoemulsification and in-the-bag intraocular lens (IOL) implantation with multiple, intertwined, discrete, pigmented cords in the anterior chamber (Fig. ). The fellow eye was phakic with best-corrected vision of 20/30 and had not undergone any prior surgeries/procedures. He did not have a history of diabetes, glaucoma, uveitis, trauma, or other intraocular surgery. Past medical history was significant for atrial fibrillation, Raynaud’s syndrome, and B-cell CLL previously treated initially with rituximab and chlorambucil, and more recently with ibrutinib for 6 months prior to cataract extraction. The lens had 2–3+ nuclear sclerosis without pseudoexfoliation or phacodonesis, and did not require mechanical pupil expansion. A retrobulbar block of 2% lidocaine and 0.75% Marcaine (bupivacaine) was administered preoperatively. No intracameral or intravitreal medications were used. At the end of the case, dexamethasone and cefazolin were applied to the ocular surface.
At presentation, his vision was 20/100 and intraocular pressure (IOP) was 43 mmHg. There was no hypopyon, hyphema, significant corneal edema, or cellular reaction. The dilated fundus examination was unremarkable. Fibrinoid syndrome was suspected. He was started on topical prednisolone every 2–3 hours, brimonidine three times per day, timolol-dorzolamide two times per day, and orally administered acetazolamide. Within 2 weeks, the cords disappeared completely (Fig. ), vision improved to 20/30, and the IOP normalized off all medications.
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pmc-6238377-1
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A 66 years old woman, presenting increasing asthenia, revealed at peripheral blood count a severe anemia (Hb 7.3 g/dL), a reduced white blood cell (WBC) count (2400/μL) with severe neutropenia (neutrophils 600/μL), and a normal platelet count (PLT 168.000/μL). The diagnostic work-up showed a B-ALL, with normal karyotype, negative for BCR-ABL rearrangement and with immature B-cell origin (CD19+, CD22+, SMIg+, TdT+, CD20-). The CT-scan performed at diagnosis revealed a solid-lesion (7.0 cm width) at the right kidney’s inferior pole, that turned out to be a clear cell carcinoma (surgically removed later). The patient received 6 courses of chemotherapy according to BFM schedule, following local Institutional guidelines, including monthly intrathecal central nervous system (CNS) prophylaxis. After the first chemotherapy cycle, she reached a morphologic complete remission (CR) with MRD negativity, evaluated by analysis of clonal rearrangement of IgH gene study (according to Biomed EuroMRD Protocol []). Such a deep response was confirmed and maintained during all the six courses of chemotherapy.
After about 1 year and a half of sustained MRD negativity, blasts were documented at the peripheral blood smear. She performed a 18F-PET/CT (PET-CT), considering the recent history of renal cancer, that documented the presence of multiple lesions (Fig. ) [], including a large pancreatic one. In order to define the following therapeutic approach, a differential diagnosis between renal cancer metastasis and EM-ALL localization was required. A pancreatic eco-endoscopic biopsy was performed, revealing a population of CD19+ and CD22+ lymphoid cells (Fig. ). Blast cells’ CD22 positivity suggested an approach with IO, which was obtained as compassionate use. IO was administered weekly in hospitalized regimen, for a total of three infusions (1,3 mg on day 1; 0,8 mg on day 8 and 15). Therapy was well tolerated, and no adverse events occurred. As expected, the bone marrow evaluation showed a morphologic CR, even with MRD positivity (10-3). EM disease was still present, though, as detected by a PET-CT scan, which showed a slight reduction of the pancreatic lesion previously reported, with the onset of new hypermetabolic areas (Fig. ). Nevertheless, a second course of weekly IO was administered, for a total of four infusions (0.8 mg per dose). Surprisingly, the further PET-CT-scan documented a complete metabolic response (CMR) (Fig. ) associated with bone marrow MRD negativity. Currently, the patient is in good clinical conditions and still on IO (course 4), waiting for the identification of a matched-unrelated donor, not yet available, to proceed to allogenic bone marrow transplantation (allo-BMT).
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pmc-6238377-2
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A 67 years old man, suddenly presented muco-cutaneous bleedings associated with severe thrombocytopenia (PLT 13.000/μL), mild anemia (Hb 8.3 g/dL) and marked hyperleukocytosis (WBC 63.000/μL, 93% blast cells). The bone marrow examination showed a remarkable lymphoblast infiltration (TdT+, CD79a+, CD22+, CD19). Conventional cytogenetic analysis revealed t(9;22). Therefore, the diagnosis of BCR-ABL1 (p190)-positive B-ALL was made. The patient received a 7-day steroid pre-phase followed by ponatinib (Iclusig) at the initial dose of 15 mg daily, rapidly increased to standard dosage, 45 mg/daily, associated with monthly medicated lumbar punctures (methotrexate, cytarabine and dexamethasone), according to the GIMEMA LAL1811 clinical trial (NCT01641107). He obtained a morphological CR after 10 days of ponatinib, while MRD evaluated by real-time PCR never reached values below 0.003 copies (assessed by BCR-ABL/ABL ratio). Therapy was well tolerated and continued for 18 months, until the patient presented a painless skin lesion on the forehead, that turned out to be a CD19+ CD22+ EM-ALL localization. Bone marrow was still negative for leukemic infiltration. After local radiotherapy (4000 cGy) the skin lesion completely resolved, but the PET-CT scan control, performed just 2 months later, revealed multiple new hypermetabolic thoracic and abdominal lesions. Therefore, the patient received two courses of chemotherapy, according to BFM schedule. Therapy was complicated by an episode of gastrointestinal bleeding (melena with severe anemia) caused by duodenal and gastric disease localizations, documented bioptically (Fig. ). The PET-CT scan after the chemotherapy courses (Fig. ) showed a remarkable disease progression (PD). Immunohistochemistry of duodenal biopsy had showed CD22 positivity, suggesting a possible efficacy of IO therapy, obtained as compassionate use. He underwent two IO courses according to standard schedule without the occurrence of any adverse event. The following bone marrow analysis documented a persistent morphological and cytogenetic CR, but still MRD positivity (BCR-ABL/ABL 0.03 copies). The PET-CT scan showed the progressive decrease of all previously described lesions (Fig. ), reaching a partial metabolic response (PMR), because of the persistence of a pathologic hypermethabolic areola. The patient proceeded to allo-BMT 1 month later. No GVHD occurred, and no VOD/SOS (Veno-occlusive disease/Sinusoidal occlusive syndrome) was observed. To sum up, the patient had several EM “outbreak” relapses, while the bone marrow morphologic complete remission with MRD positivity was maintained. None of the therapies he underwent (TKI, local radiotherapy, chemotherapy, IO) lead to MRD negativity. However, notably, only IO induced an important reduction of extramedullary disease localizations.
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pmc-6238405-1
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A 74-year-old male, who had undergone 3 stent coronary implantation procedures in the previous 6 months, presented to the hospital with progressive dyspnea and recurrent chest pain. The patient’s medical history was noted for esophageal cancer treatment that consisted of a radical esophagectomy, gastric pull-up followed by chemotherapy and radiotherapy. Esophageal cancer recurrence was ruled out. A transthoracic echocardiography revealed severe aortic regurgitation, moderate mitral regurgitation and a left ventricular ejection fraction of 44%.
The patient underwent a dual valve replacement procedure with a bioprothesis aortic valve (23 mm Magna Ease, Edwards Lifesciences, CA, USA) and mechanical mitral valve (25 mm ON-X, CryoLife, GA, USA). The patient required 7 days of inotropes and intensive unit care. On postoperative (PO) day 8, a right-sided chylothorax was diagnosed, and treated with simple drainage and low-fat medium chain triglycerides diet. On PO day 18, the patient evolved with acute respiratory deterioration and hypoxemia. Chest auscultation revealed peristaltic sounds on the left side. Chest x-ray revealed right pleural effusion and abdominal contents within the left chest cavity (Fig. a). Despite pleural effusion drainage, the patient had only slightly improved the respiratory status (Fig. b). A chest computerized tomography confirmed the presence of a large portion of the transverse and descending colon in the left hemithorax with no radiological sign of intestinal necrosis (Fig. ). The diaphragmatic hernia measured 15 cm and filled the whole transverse dimension of the left chest on the anterior-posterior view. A transthoracic echocardiogram ruled out acute cardiac complications. Clinical deterioration was evidenced by increased oxygen requirements to 5 L/min, tachypnea, tachycardia and confusion.
Urgent diaphragmatic hernia repair was indicated and performed by laparoscopy. The patient was placed in a dorsal position with hyperextension of the upper third of his abdomen. Laparoscopic surgery was performed through two 12 mm trocars on the left and right paraumbilical region and three 5 mm trocars were used in the subcostal region, one on the right side and two on the left. The 10 mm 30G camera was inserted through the left paraumbilical incision. A large quantity of peritoneal adherences was taken down with harmonic synergy blades (Ethicon, OH, USA) under direct vision. A large diaphragmatic hernia was identified with a large portion of the transverse colon and omentum within the left chest cavity. Once the majority of the colon was reduced the dissection of the hernial sac from the right and from the left hiatal pillars toward the mediastinum and the apex of the left chest. Pealing the sac off was mandatory for a complete reduction and repair of the hernia. As expected, the apical portion was the most laborious however with proper exposure the dissection was safely performed. The colon stayed passively in the abdominal cavity. The repair of the hiatal hernia was performed by approximating the left and right pillars with non-absorbable stiches. A Biodesign Hiatal Hernia Graft (Cook Medical, IN, USA) was placed surrounding the hiatus. A prolene mesh (Ethicon, OH, USA) was used to close the anterior space. Both grafts were fixed with tacker fixation device (Medtronic, MN, USA). The final result was satisfactory.
The patient had postoperative ischemic colitis and interstitial alveolar left lower lobe infiltrate. This was managed with a conservative treatment based on antibiotics and parenteral nutrition. A Pleur-X chronically indwelling catheter system (Becton Dickinson, NJ, USA) was installed in the patient before discharge, 3 months after surgery. One year after discharge, the patient was readmitted with increased dyspnea. A right sided chylothorax, secondary to Pleur-X infection, was diagnosed. The drainage system was changed, antibiotic treatment was given for 2 weeks and the patient is now doing well.
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pmc-6239614-1
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Our patient is a 53-year-old woman with a posterior, stage IIB NSCLC in the left upper lobe who received neoadjuvant carboplatin, pemetrexed, and radiotherapy. One year following diagnosis, the patient was found to have tumor attachment to the T2 and T3 vertebrae (Figures - ) and infiltration of the corresponding nerve roots following complaints of severe left back pain and left axillary numbness and paresthesia. As a result, the neurosurgery and thoracic surgery services were consulted to discuss management. Based on the magnetic resonance imaging (MRI) studies, there was no direct tumor invasion of the left brachial plexus or subclavian vessels. After discussing different treatment options, a posterior approach, image-guided T2 and T3 osteotomy followed by transthoracicen blocresection of the thoracic tumor was recommended. The patient agreed to the operation and informed consent was obtained.
After the initial incision was made in the operating room, we exposed the spinal processes, lamina, and transverse processes from T1 through T5 and 5 cm of the ribs from the left costovertebral junction on the side of the tumor. At this point, images were taken with the O-Arm(Medtronic Corporation, Minneapolis, Minnesota, US). We then registered the patient's spine to the StealthStation (Medtronic Corporation, Minneapolis, Minnesota, US) navigation suite using the stereotactic probe. Then, using spine navigation, the instrumentation was placed at the level above and below the tumor-infiltrated vertebrae. We did not place screws on the left T2 and T3 vertebrae (Figure ). The spinous processes and lamina of T2 and T3 were removed and the nerve roots exposed on the left. We noticed an infiltration of the ganglia by the primary tumor that was in continuity with the ribs on the left side. We then proceeded with resection; the T2 and T3 nerve roots were tied with a 2-0 silk tie and cut 2 mm from the tie on the extradural portion of the root. On the spine-navigation station, we determined the plane of osteotomy of the vertebral bodies of T2 and T3 (Figure ). We anticipated disconnecting the portion of the vertebral body infiltrated by the tumor from the rest of the vertebral body, leaving it in situ for removal with the entire lung tumor in the second stage of the procedure. During the planning of our trajectory, we considered that the periosteum of the vertebrae had been infiltrated. We wanted to resect starting from the point where the tumor had infiltrated the nerve root projecting then on the anterior portion of the vertebra, planning a resection margin free of tumor from the infiltrated periosteum. Using an ultrasonic Sonopet with a serrated knife bone cutter tip (Stryker Corporation, Kalamazoo, Michigan, US), the osteotomy of the vertebral bodies of T2 and T3 was performed following the planned trajectory with the intent to leave the mass intact and avoid entering the tumor capsule. As the osteotomy was performed, we intermittently used the navigation probe to confirm our trajectory. We continued cutting through the bone with the Sonopet blade until soft tissue was felt underneath. The location was then confirmed with stereotactic spine navigation images (Figure ). Here, we proceeded with cutting the second and third ribs 5 cm from the midline. After we confirmed that the ribs, together with the portion of the vertebral body wall infiltrated with tumor and the nerve root, were completely freed posteriorly, we proceeded with standard arthrodesis. We separated the muscle from the fascia to mobilize the tissue and closed the tissue with layered sutures. Following closure, the en bloc resection of the left upper lobe of the lung with the lateral portions of the T2 and T3 vertebral bodies and corresponding ribs together with complete mediastinal lymphadenectomy was performed through a posterolateral standard thoracotomy incision (Figures -).
The final surgical pathology report revealed moderately differentiated, acinar predominant stage IIB adenocarcinoma with a single focus, all three resected lymph nodes, and six margins were negative for tumor. The nearest margin was 4 mm from the tumor, confirming the accuracy of our planned trajectory on the StealthStation. One year later, the patient was doing well, with no evidence of tumor recurrence. The patient declined any further therapy during that time and was subsequently lost to follow-up.
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pmc-6240021-1
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A 90-year-old woman visited our hospital due to a large area of erythema and localized skin ulceration with hemorrhage of her right breast. Her breast symptoms arose 5 years ago and had been worsening. She could not visit a hospital because she expected for naturally healing and feared noticing cancer and death. She had a history of atrial fibrillation and cerebral infarction 2 months earlier, then her breast lesion was found out. The area of erythema was 15 × 15 cm2. Her nipple and alveolar complex were destroyed and had an uncertain shape. Her skin erythema was soft, and no tumor was palpable (Fig. ). Her quality of life had got worse by hemorrhage and exudate from the tumor, and she felt strong anxiety about getting more worse and death from the cancer.
A punch biopsy indicated mammary Paget’s disease. Computed tomography showed that the tumor was only on the surface of the breast, with no metastasis including of the axillar lymph nodes. There was no underlying tumor in the breast (Fig. ).
Even though our patient was a very elderly woman with comorbidities and her prognosis was relatively good, her symptoms were intolerable. By the request of her and her family, we decided to perform surgery to eliminate the area of erythema after receiving sufficient informed consent. The surgical treatment was performed by two teams that included surgeons and dermatologists. We drew a resection line 1 cm from the skin erythema. Dermatologists were on standby in case a skin graft was needed. We performed muscle-sparing mastectomy with sampling of an axillar lymph node. We added two stress-relaxation sutures to avoid diastasis because the excision area was very large and the tension of the skin flap was strong (Fig. a, b). Fortunately, a skin graft was not necessary and her postoperative course was good. The skin flap did not develop major complications such as necrosis, seroma, wound infection, and highly disturbance of moving the right upper limb. We removed the stress-relaxation sutures 7 days after surgery.
A histological examination revealed mammary Paget’s disease without invasion to underlying tissues (Fig. ), no evidence of a residual tumor of the entire stumps, and no metastasis in the lymph node. Although she felt a little tightness of the surgical site, paresthesia of the chest wall, and a sense of breast loss, her quality of life improved after surgery by being freed from symptoms and anxiety related to malignancy. It was a great value for her, even if she suffered from these complications.
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pmc-6240022-1
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A 90-year-old man had been followed by a cardiologist because of diabetes mellitus, chronic renal failure, and an abdominal aortic aneurysm. A solid mass was found on plain computed tomography (CT) at a regular health check-up. He had smoked 20 cigarettes per day for 45 years. Plain CT showed a solid mass, 31 mm × 28 mm, with a partially unclear margin with the normal thymic tissue in the anterior mediastinum (Fig. ). Magnetic resonance imaging (MRI) showed an iso-intensity mass on T1-weighted images and high intensity on T2-weighted images (Fig. ). Diffusion-weighted imaging showed a high-intensity area in the marginal zone, with apparent diffusion coefficient sequences. Laboratory findings and results for markers such as alpha-fetoprotein, beta-human chorionic gonadotropin, anti-acetylcholine receptor antibody, and soluble interleukin-2 receptor were not significant preoperatively. 18F-Fluorodeoxyglucose-positron emission tomography (FDG-PET) showed the mass had marked uptake of FDG, early maximum standardized uptake value (SUVmax) of the mass 30.5 (Fig. ). The mass was thought most likely to represent thymic cancer, followed by invasive thymoma, Masaoka stage II, and UICC-T1bN0M0 stage I. First, video-assisted thoracic surgery (VATS) was tried through the left pleural cavity. Strong and broad adhesions between the left lung and the chest wall were observed. Since VATS appeared risky, the procedure was converted to median sternotomy. An anterior mediastinal tumor was fixed to the anterior chest wall. We attempted dissection in the extrapleural layer, but the tissue was not easily dissected. The tumor seemed to be invading into the left upper lobe of the lung and the chest wall. We abandoned dissection at once. Partial thymectomy, with combined partial resection involving left upper lobectomy and the first and the second costal cartilages, was done. Operation time was 4 h and 29 min, and blood loss volume was 450 ml. The patient’s postoperative course was uneventful. Histopathologic examination showed a white, solid, 35 × 30 × 25 mm3 mass with regional bleeding and necrosis (Fig. ). Microscopically, the tumor nests composed of atypical cells with large nuclei showed a palisading or organoid pattern. Cells with bizarre or multiple nuclei were also seen. Forty-fifth mitoses per 2 mm2 and broad necrosis were seen. The surgical margin was free from tumor cells. Immunohistochemistry showed positive staining for chromogranin A, synaptophysin, and CD56 and negative staining for CD5 and p40. The tumor cells also showed positive nuclear staining for thyroid transcription factor-1 (TTF-1). Histology proved the tumor invasion to the left upper lobe of the lung but not to the costal cartilage. Most of the lesion was located not in the lung, but in the mediastinal fatty connective tissue. We thoroughly observed the running of a pleural elastic layer by elastic fiber staining (Elastica van Gieson). The elastic layer of the visceral pleura bent in the way to be convex in the lung near the marginal part of the tumor, and its running manner became intermittent as it reached toward the center of the tumor, which finally disappeared. We could consider this may indicate that the primary anterior mediastinum tumor invaded into the lung. The final pathologic diagnosis was thymic LCNEC, Masaoka stage III, and T3N0M stage IIIA. Five months after surgery, CT showed pleural dissemination and left lung metastasis. The patient was given palliative care and died of the original disease 12 months after surgery.
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pmc-6240177-1
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A 20-year-old Chinese man with no significant medical history was referred for sudden headache with diplopia. His sudden headache started in July 2012 and was aggravated over 3 months by fatigue, recurrent fever, nausea and weight loss, followed by bilateral vision loss and intermittent diplopia. His body temperature was repeatedly elevated, with a maximum temperature of 39.7 °C. His best-corrected visual acuity (BCVA) was 10/20 bilaterally, with a normal intraocular pressure (IOP). He showed ptosis in both eyes, with restricted abduction on the right side. A slit-lamp examination yielded normal results for both the anterior segment and the fundus, with no relative afferent pupillary defect (RAPD). VF testing revealed bitemporal hemianopsia. Laboratory tests showed a white blood cell (WBC) count of 6.26 × 109/L and a neutrophil count of 3.73 × 109/L (59.5%). His renal function was normal, with a creatinine (Cr) and urea level of 61.17 μmol/L and 4.05 mmol/L, respectively. The urine was negative for protein and red blood cells. The urine-specific gravity was normal, while endocrine tests revealed a thyroid-stimulating hormone (TSH) level of 0.04 μIU/mL, an adrenocorticotropic hormone (ACTH) level of 1.70 pg/mL and a testosterone level of < 20.0 pg/mL. Morning cortisol, prolactin (PRL), random blood glucose and glycosylated hemoglobin levels were normal (Table ). Enhanced MRI showed pituitary enlargement with increased T2 signal intensity and heterogeneous enhancement. The sellar mass displayed a suprasellar extension and optic chiasm compression, along with bilateral extension into the cavernous sinus (Fig. and ). No abnormalities were found by chest or abdominal computed tomography (CT) or in the levels of tumor markers, C-reactive protein (CRP), antistreptolysin O (ASO) or rheumatoid factor (RF). The immune test results were negative for ANCAs (myeloperoxidase [MPO]-ANCAs, 3.89 RU/mL; proteinase 3 (PR3)-ANCAs, 3.09 RU/mL; reference interval, < 20 RU/mL), as well as antinuclear antibodies (ANAs) and anti-extractable nuclear antigen (ENA) antibodies. The total serum IgG level was 12.30 g/L (7.00–17.00), with an IgA level of 2.01 g/L (0.70–4.00) and an IgM level of 0.35 g/L (0.40–2.30). The patient was suspected to have immune-related pituitaritis. The cerebral spinal fluid (CSF) was then tested. The results indicated a WBC count of 13*106/L and an IgG level of 4.63 mg/dL in the CSF. IgG oligoclonal bands were absent in the serum and CSF, which had no traces of bacteria, such as Staphylococcus aureus and Mycobacterium tuberculosis. A CSF smear showed a mass of lympho-monocytes and macrophages. The patient was then diagnosed with lymphocytic hypophysitis and was treated with IV dexamethasone (20 mg qd) for 3 days, followed by a decreased dosage of dexamethasone (10 mg qd*7 days, 5 mg qd*2 days) and then oral prednisone (60 mg qd). Two months later, the patient’s BCVA recovered to 100/100 bilaterally with a normal VF. His ocular movement was normal, and he reported no diplopia or headache. Additionally, the endocrine hormone levels were within normal limits (Table ). A repeat enhanced MRI showed that the pituitary mass was smaller than before with homogeneous enhancement, and the chiasmal compression had diminished (Fig. and ). The patient’s condition remained stable during the following year, with no significant changes observed by MRI.
In Sept. 2014, he was readmitted to the local hospital due to headache recurrence with nausea and vomiting. Endocrine tests showed an elevated PRL level and hypothyroidism (Table ). Repeated serum immune tests yielded negative results for ANAs, MPO-ANCAs and PR3-ANCAs. Enhanced MRI revealed pituitary enlargement with stalk compression and chiasmal thickening (Fig. ), indicating recurrent lymphocytic pituitaritis, which was treated with oral corticoids (60 mg qd). The endocrine hormone levels returned to normal, but the headache was not relieved.
In Oct. 2014, the patient’s headache worsened with severe nausea and vomiting, and the visual acuity in his right eye decreased to hand motion (HM), with 80/100 in the left eye and an IOP of 13/17 mmHg. His left eye displayed ptosis, but the ocular position and eye movement were normal. A slit-lamp examination showed no abnormal findings in the anterior chamber, with an equal pupil size, but the right eye was RAPD positive. The fundus examination was normal except for bilateral pale optic papillomas (Fig. ). VF testing revealed total blindness in the right eye and temporal hemianopsia in the left eye (Fig. ). Optical coherence tomography (OCT) showed a significant decrease in the thickness of the retinal nerve fiber layer. Repeated enhanced MRI showed pituitary enlargement and a new CNS lesion with abnormal nodal T1 and T2 enhancement on the right side of the suprasellar region; the lesion was invading the pituitary stalk, infundibulum, right optic nerve, posterior right basal gyrus rectus of the frontal lobe, and anterior perforated substance and extending to the internal carotid artery (Fig. ).
Erythrocyte sedimentation rate (ESR), CRP, cryptococcal antigen, serum 1,3-beta-D-glucan assay (BDG test), interferon gamma release assay for tubuerculosis (T-SPOT test) and a lymphocyte culture yielded negative results. An enhanced paranasal CT scan showed only bilateral ethmoid and left sphenoid sinus inflammation. No positive results were detected by chest X-ray or multiple-organ B-mode ultrasound examination. Moreover, there were no traces of red blood cells or protein in his urine. The patient’s renal function was also normal (urea, 5.03 mmol/L; Cr, 58.04 μmol/L). A multidisciplinary consultation concluded that with 30 days of oral corticosteroid therapy and no signs of relief, a CNS infection should not be excluded; therefore, oral prednisone (35 mg qd) was continued. The visual acuity of the patient’s right eye decreased to NLP 6 days later, with headache aggravation, sudden nausea and vomiting, and a reduction in the visual acuity of the left eye to counting fingers (CF). An ophthalmological examination, including an assessment of eye position and movement and the anterior and posterior segments, yielded the same results as before. Repeated VF test showed a temporal hemifield and a superior nasal quadrant defect (Fig. ). Repeated enhanced MRI showed meningeal linear enhancement. Two days later, the visual acuity in his left eye decreased to NLP with bilateral pupil mydriasis and disappearance of the light reflex. The results of a biopsy conducted in Dec. 2014 suggested GPA (see the section). The patient was treated with IV methylprednisolone (500 mg qd for 3 days, followed by 250 mg qd for 3 days and then 125 mg qd for 3 days). He claimed complete headache and left proptosis remission but showed no improvement in the bilateral visual acuity or pupil reflex. MRI showed a significant reduction in the parenchymal and chiasmal edema (Fig. and ). The patient’s pituitary biopsy confirmed the pathological manifestation, but repeated tests showed negative results regarding hematuria, proteinuria, and renal function and no abnormalities on chest X-ray or paranasal sinus CT. He did meet one of the 1990 American College of Rheumatology (ACR) GPA diagnostic criteria. However, considering his biopsy results and excellent response to corticosteroid therapy, we considered the diagnosis to be GPA with isolated pituitary involvement. The patient and his family requested the cessation of treatment and refused immunosuppressive therapy. 15 months after treatment with oral prednisone starting at 60 mg qd and decreasing by 5 mg every two weeks, he showed no signs of recurrence (May 2016).
The pituitary tissue specimen was stained and tested at two institutions in China (Sanbo Neurology Hospital and Peking Union Medical College Hospital), with similar results. Repeated acid-fast staining showed negative results. Hematoxylin and eosin (H&E) staining (Fig. ) showed a normal arrangement of acinar cells with scattered Langerhans cells, giant cells, and large numbers of lymphocytes and plasma cells, indicating granulomatous inflammation. Small blood vessels showed fibrinoid necrosis with neutrophilic and lymphocytic infiltration. The pathological diagnosis was GPA. The immunohistochemical staining results were positive for CD-200 and CD-68. The rate of IgG4-positive staining was approximately 20% (Fig. ).
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pmc-6240209-1
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A 48-year-old female presented with a 3.7 × 1.9 cm mass deep to the deep lobe of the parotid gland, with extension into the parapharyngeal space. Fine needle aspirate (FNA) of the lesion revealed malignant cells, but without a specific diagnosis. Surgical excision was carried out in the form of a transmandibular approach to facilitate subtotal parotidectomy, neck dissection, and resection of tumor at the skull base.
The tumor demonstrated a high-grade carcinoma with extensive perineural invasion and positive neck nodes. Original pathological diagnosis was of adenocarcinoma NOS of salivary gland origin. Thus, the patient underwent adjuvant radiotherapy in the form of 60 Gy in 30 fractions to the tumor bed, retropharyngeal lymph nodes, and left neck. An additional 10 Gy over five fractions was administered to the tumor bed and retropharyngeal nodes.
Four years following initial treatment, the patient returned to the otolaryngology clinic with a peri-incisional lesion originally thought to be a traumatic neuroma. Excisional biopsy was performed and revealed malignant cells. Immunohistochemistry was in keeping with her previous parotid cancer, confirming regional metastasis. Secretory carcinoma was suspected and subsequently confirmed by FISH analysis.
During this same time period, surveillance CT revealed a 0.7 cm lung nodule. This lesion would unfortunately expand to 0.9 cm in size. The patient underwent microcoil guided thoracoscopic wedge resection. Pathology of this lesion demonstrated metastatic SC, with negative margins but positive for vascular invasion. Serial surveillance CT scans were chosen in lieu of systemic chemotherapy. The patient has done well following metastasectomy, with no further evidence of recurrence or metastasis, now ten years from her original diagnosis and 5.5 years from her metastasectomy.
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pmc-6240213-1
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A 67-year-old female patient presented in the emergency department with persistent chest pain for 12 h, followed by aggravating lower limbs numbness and oliguria, with a urine output of 40 ml after onset. Computed tomography angiography (CTA) revealed an acute type B aortic dissection with a primary entry tear approximating to the left subclavian artery (LSA) and extending to the iliac arteries (Fig. a). Left renal artery originated from a severely stenotic true lumen, right renal artery with dynamic occlusion was supplied via a false lumen (Fig. b), and there were two cysts (28 mm × 25 mm, 10 mm × 10 mm) separately located at the upper and inferior poles of right kidney (Fig. c). Incomplete thrombosis was detected in the bilateral common iliac arteries (Fig. d).
The patient underwent emergent TEVAR 3 h after admission because of the malperfusion symptoms of right kidney and lower extremity. A hydrophilic angled guidewire (0.035 in. × 180 cm; Radifocus, Terumo) was inserted into the aortic true lumen via the right femoral artery, and the angiography showed poor perfusion of the right kidney and bilateral iliac arteries (Fig. e). The distal restrictive covered stent (straight 24 mm × 80 mm; Endurant, Medtronic) was introduced and deployed at the proximal descending aorta. Subsequently, the thoracic stent graft (straight 36 mm × 200 mm; Valiant Captiva, Medtronic) was introduced, overlapped 30 mm with the restrictive stent and deployed at the distal aortic arch (Additional file ). The LSA was sacrificed because of inadequate proximal landing zone and the dominant right vertebral artery. Completion angiography demonstrated a satisfactory coverage of the primary entry tear, and the reopening of the distal true lumen and an improved flow in right renal artery and bilateral iliac arteries (Fig. f).
Although the distal malperfusion syndrome was successfully treated, the patient showed hemodynamic instability and progressively decrease of hemoglobin from 118 to 82 g/L within 5 h after surgery. Bedside ultrasonography and abdominal CTA revealed a massive right perinephric hematoma measuring 10 cm × 15 cm (Fig. a, b). As her vital signs were unstable even after 6 units of blood transfusion and another 2000 ml of fluid resuscitation within 4 h, an emergency transcatheter embolization was performed. The right renal angiography detected multiple tortuous vascular branches with diffuse perinephric bleeding (Fig. c). The main trunk of right renal artery was embolized with metallic microcoils (0.035 in.; Cook) and histoacryl glue (B. Braun) (Fig. d, Additional file ).
The patient was hemodynamically stable, and the hemoglobin returned to normal. Although a period of renal insufficiency with the maximum serum creatinine level reaching 343 μmol/L was observed after surgery, the patient maintained normal urine output (1200-1500 ml/day) without any hemodialysis. The patient recovered uneventfully, and no signs of hemorrhage of the right kidney were detected by pre-discharge CTA 7 days after TEVAR (Fig. e, f). Six-month follow-up showed the patient was in good condition and presented with normal renal function.
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pmc-6240213-2
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Another patient was a 69-year-old male who was admitted for endovascular repair of a chronic complicated type B aortic dissection. He had history of poorly controlled hypertension for 10 years due to irregular intake of antihypertensive medications. The dissection ranged from the distal aortic arch to the iliac arteries (Fig. ). The left renal artery originated from the true lumen, and the right renal artery was supplied via both the true and false lumen, no cysts or tumors were found in both kidneys (Fig. ).
TEVAR was accepted as a reasonable treatment strategy after the consent of the patient. Through right femoral artery, the first (distal) (tapered 28 mm–24 mm × 150 mm; Valiant Captiva, Medtronic) and the second (proximal) (straight 34 mm × 200 mm; Valiant Captiva, Medtronic) thoracic stent grafts were accurately deployed without sacrificing LSA, and the entry tear was successfully occluded (Fig. , Additional file ).
The patient complained of left flank pain and presented with hemodynamic instability early after TEVAR. His systolic blood pressure rapidly decreased to less than 80 mmHg, and the hemoglobin value sharply dropt from 122 to 64 g/L within only 2 h postoperatively, even intravenous bolus administration and massive blood transfusion could not maintain his vital signs stable. Bedside ultrasonography showed a giant left retroperitoneal hematoma. Progressively hemodynamic instability forced cessation of further radiological examination. The patient was immediately transferred to the operating room for emergency transcatheter embolization because of highly suspicious of left RH. The abdominal angiography revealed two active bleeding sits located in the distal branches of left renal artery, and no bleeding sites were found at the aorta and other branches (Fig. , Additional file ). A super-selective embolization of two renal arterial branches was performed with metallic microcoils (0.018 in.; Cook) and histoacryl glue (B. Braun). Completion angiography showed effective occlusion of the feeding vessel and termination of the left renal bleeding (Fig. , Additional file ). The patient developed abdominal compartment syndrome and died of multiple organ failure 2 days after surgery.
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pmc-6240253-1
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Fifty-seven-year-old woman.
BMI: 27 kg/m2 (155 cm, 65 kg).
Difficulty in urination.
The patient suffered from a symptom having difficulty in urination from June, 2016. When she visited a local gynecology outpatient clinic due to abdominal discomfort on July 12, 2016, a huge uterine mass (heterogeneous 12 cm) was detected on the left through an ultrasound. Then, she was transferred to the department of Gynecology of Pusan National University Yangsan Hospital. A blood test conducted on July 13, 2016 showed that CA125 was 2543.1 U/mL, HE4 was 1361.6 pmol/L, and ROMA (postmenopausal) was 99.4261. In a subsequently conducted genetic test, BRCA 1, 2 turned out to be negative. A Pelvis CT conducted on July 15, 2016 showed a finding of suspected primary ovarian cancer (Fig. ). Then, she underwent exploratory laparotomy (TAH, BSO, BPLND, PALND, Appendectomy, Omentectomy) on July 25, 2016. Finally, she was diagnosed with ‘ovarian cancer, serous carcinoma 1C, grade 1’ through a frozen biopsy. The patient refused to receive postoperative anticancer treatments and her condition has been monitored at the hospital outpatient department without any findings of a recurrence of the illness. On June 5, 2017, she visited the Occupational and Environmental Medicine outpatient clinic of Pusan National University Yangsan Hospital for work-relevance evaluation. At that time, she also had as symptom difficulty in breathing, accompanied with dry cough. Pulmonary function tests and chest X-ray showed no specific findings but through a chest CT conducted on June 20, 2017, she was diagnosed with suspicious asbestosis along with a finding of pleural plaque (Fig. ).
Parity 4–2–2-2. She married at the age of 23 and became the first full-term pregnant woman at the age of 25. All of her children were born with virginal delivery. She has breastfed all children for over a year. The patient underwent a sterilization operation when she was 29 years old. and reached menopause when she was 55 years old. She did not take any hormone replacement therapy after menopause. There was no special cancer history. A Pap smear test conducted in June, 2016 turned out to be negative.
Non-smoker, social drinker (3 standard drinks per week). No issues were found in the family history and medication history. Her husband worked for about 4 years as an automobile parts production worker.
The patient performed chrysotile twisting and spinning works for one year and two years and seven months, respectively, at an asbestos textile factory from March, 1976 to October, 1979. She was sometimes sent to crocidolite part 1–2 times a month short for 2–3 days, long for 1 week, and usually worked a double shift day and nights for six days a week (08:00–19:00 in the daytime, 19:00–08:00 in the night time) but when there were a lot of work load, she worked for even seven days a week. At that time, workplaces were furnished with an air exhauster and workers wore a dust mask, but the levels of asbestos dust were very high since asbestos fibers easily broke into small particles during the process of spinning. Indeed, a thick layer of white dust sat on the scalp of workers even if they were hooded. As for working clothes, she washed them at home after work. She left the asbestos factory after marriage and then has been engaged in the restaurant business.
The asbestos factory where the patient worked was operated from 1969 to 1992 []. She lived around 1 km away from the factory before 1973, around 500–1000 m away from 1973 to 1982, and around 3.5 km away from 1982 until now.
Since the asbestos factory no longer exists, levels of exposure to asbestos at the asbestos textile factory at that time were estimated through a literature review [, –]. Now that records on levels of exposure to asbestos in the 1970s when the patient worked are hardly left, the review was focused on the exposure to asbestos during the period most similar to her working period, among previous literature on levels of exposure to asbestos in the past. Among several asbestos textile process steps, levels of exposure during twisting and spinning process steps, in which the patient was involved, were estimated and the exposure values estimated through literature were considered to be the minimum exposure levels of the worker. The maximum exposure concentration based on Korean measurement data was confirmed to be 45.8 fiber/cc, which was measured during the weaving process at an asbestos textile factory in 1987. The levels of exposure to asbestos in the air at an asbestos textile factory from 1984 to 1992 are as shown in Table [–, ]. With regard to exposure levels by year, the earlier the time was, the higher the exposure level was. The weighted mean of exposure levels at the measured workplaces was confirmed to be 3.58 fiber/cc. Levels of exposure to asbestos showed a big difference depending on process step and type of works even within the asbestos textile industry. According to the data released by the Korea Occupational Safety & Health Agency (KOSHA) in 2006, exposure levels by process step in the asbestos textile industry are as shown in Table []. If the maximum exposure levels during twisting and spinning process steps (14.9 and 15.0 fiber/cc, respectively) are applied to the patient, levels of exposure to asbestos are 14.9 fiber·year/cc (14.9 fiber/cc × 1 year) and 38.7 fiber·year/cc (15.0 fiber/cc × 2.58 year), respectively. If the geometric mean of exposure levels in 1987, most similar to her working period, is applied, levels of exposure to asbestos are 4.8 fiber·year/cc (4.8 fiber/cc × 1 year) and 14.45 fiber·year/cc (5.6 fiber/cc × 2.58 year), respectively. In conclusion, it is presumed that the patient was exposed to 19.25 or 53.6 fiber·year/cc of asbestos or more while working at an asbestos textile factory for three years and seven months.
Based on the patient’s residential history, non-occupational exposure to asbestos was assessed through the method proposed by Magnani []. Given that she lived around 500–1000 m away from the asbestos textile factory for about 10 years from 1973 to 1982, the environmental exposure is applicable to high probability and middle intensity. In addition, considering that she brought her working clothes home and washed them at home, the domestic exposure is applicable to high probability and high intensity.
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pmc-6240271-1
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A 33-year-old man presented with right facial palsy and right hand fine motor dysfunction for over previous 3 months. MRI revealed a gadolinium-enhanced mass lesion in left basal ganglia with extension to crus cerebri and left thalamus (Fig. ). Stereotactic biopsy was performed and the lesion was identified as glioblastoma, IDH-wild type.
The past medical history revealed the patient was once diagnosed with pineal mass for almost 20 years ago in 1995, when he was a 12-year-old boy. At that time, he underwent total removal of tumor via the right occipital transtentorial approach and biopsy identified germinoma. Postoperatively, he received craniospinal irradiation 24Gy and whole-brain radiotherapy 36Gy, that is, tumor bed total 50.4Gy. He was free from tumor recurrence or secondary tumor until 2006, when he had the last follow-up MRI. After then, he had no MRI follow-ups until the newly-developed symptoms occur in January 2015.
Although we thought that this was evidence of tumor recurrence, biopsy identified glioblastoma, and we suspected that it was a therapy-associated tumor (Fig. ). He received concurrent chemoradiotherapy with temozolomide. As tumor progression was identified during follow-up, he underwent bevacizumab/irinotecan and metronomic temozolomide consecutively till 15 months after the biopsy. But, the tumor progressed and he died in May 2016.
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pmc-6240289-1
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A 71-year-old Caucasian man presented to the emergency department of our hospital with a 1-week history of abrupt-onset blurry vision, dizziness, nausea, vomiting, and ataxia initially thought consistent with a posterior circulation stroke. The patient denied associated vertigo or headache. He had no prior history of stroke and had been taking prophylactic aspirin for years for a patent foramen ovale. Noncontrast head computed tomography (CT) performed in the emergency department demonstrated no visible masses or hemorrhage. A shrapnel adjacent to the patient’s eyes precluded the possibility of further visualization with magnetic resonance imaging (MRI). He was admitted for further workup and treatment. Carotid Doppler ultrasound showed no stenosis. Subsequent CT angiography did not clearly visualize the brain parenchyma but showed no vascular compromise. The initial working diagnosis was of a cerebellar stroke, and the patient was transferred to the acute inpatient stroke rehabilitation service.
Despite participation in rehabilitation therapies, his symptoms progressively worsened, prompting repeat noncontrast head CT 9 days after admission, which demonstrated indistinct, masslike lesions in the cerebellum, one with evidence of hemorrhage and surrounding vasogenic edema and mild hydrocephalus. Contrast-enhanced CT performed later that day revealed three intensely enhancing masses in the right cerebellar hemisphere (Fig. ). The patient was started empirically on steroids for his vasogenic edema, which produced rapid improvement in his symptoms. Because these cerebellar lesions appeared most consistent with metastatic disease, the neurosurgery service recommended metastatic cancer workup without immediate surgical intervention. CT with contrast enhancement and whole-body positron emission tomography failed to demonstrate a primary tumor of origin outside the central nervous system (Fig. ). The patient underwent right suboccipital craniotomy with partial resection of the visible tumor in the right cerebellum. Histopathology revealed diffuse large B-cell lymphoma, non-germinal center type (Figs. and ).
Bone marrow biopsy and testicular ultrasound demonstrated no evidence of lymphoma in these sites. The result of human immunodeficiency virus (HIV) testing was negative. The patient elected to pursue induction chemotherapy. Because of his age, the radiation oncology service recommended against whole-body irradiation to minimize neurotoxicity. The patient was started on the MATRix regimen (methotrexate, cytarabine, and rituximab), which the patient has tolerated well thus far, with no residual disease seen on contrast-enhanced head CT scans. The patient has experienced marked improvement in his symptoms with treatment and is able to ambulate with a walker, but he still reports balance problems and blurry vision.
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pmc-6240425-1
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Patient 1 (III:9) was a 11 years old male, who presented with a history of multiple fractures after mild trauma since the age of 6 months. His first fracture of right clavicle occurred at the age of 6 months, followed by fracture of right tibial shaft at the age of 8 years, and fracture of right and left femora at 9 years. Pregnancy and birth were uneventful. His parents were distant cousins (IBD proportion < 6%). Presently, the index individual is wheelchair bound because of multiple fractures, deformities and weakness of lower extremities. His weight is 20 kg, height 106 cm and head circumference 52 cm. Physical examination revealed brachycephalic head, flat face, mild blueness of sclerae, right eye squint, short neck, marked thinness of upper and lower extremities, anterior angulation of both femora and right tibia, and flat feet (Fig. ). Skeletal radiographs revealed generalized osteopenia, bowing of clavicles, compression of thoracic vertebrae, narrowing of intercostal spaces, and bowing of long bones.
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pmc-6240425-2
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Patient 2(III:15) was a 10 years old male, wheelchair bound, measuring 18 kg in weight, 107 cm in height and 51 cm in head circumference. Pregnancy and delivery were uneventful. Parents were also distant cousins (IBD proportion ~ 6%).His health remained poor since birth. He was able to walk but never became able to run. At the age of 8 years he sustained fractures of right humerus and right tibia and femur after trivial traumas (Fig. ). Clinical examination showed triangular face, normal teeth, right eye squint, normal sclerae, and wide protruding chest with increased antero-posterior diameter. Extremities were thin and showed bilateral mild angulation of proximal humeri and marked bowing of right femur. His feet were flat. Radiological examination showed generalized osteopenia, platyspondyli in all vertebrae, narrowing of inter-costal spaces, globular pelvis, bowing of distal parts of right tibia and fibula, and mild bowing of right radius and ulna (Fig. and ).
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pmc-6240427-1
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A 67-year-old Japanese man with a past medical history of hypertension, diabetes mellitus, and angina presented with a history of generalized weakness, lethargy, cold intolerance, weight loss, and loss of appetite. The patient was a smoker who had been smoking a half-pack per day for 47 years. His family history was unremarkable.
One and one-half months prior to admission, the patient had symptoms of lethargy and anorexia, with a 7-kg weight loss in only 1 month. On the admission day, he could not move because of overall weakness and lethargy and was transferred to our hospital. He was conscious and oriented, and his blood pressure was low (104/70 mmHg) compared with his previous hypertension. His blood glucose on arrival was low (64 mg/dl). His body temperature was 35.8 °C, heart rate (HR) was 60 beats/min, and respiratory rate was 20 breaths/min. No conjunctival pallor or thyromegaly was appreciated. Cardiac and pulmonary examination results were normal, other than a positive tilt test. His neurological examination revealed that his higher cognitive functions were normal, as were the cranial pairs, with no visual defect.
Laboratory studies revealed that the patient’s complete blood count and coagulation were normal. Biochemistry tests revealed a sodium level of 134 mEq/L (reference range, 135–147) and hypoglycemia, but the other electrolytes were within normal limits. Notably, the level of thyroid-stimulating hormone (TSH) was 0.505 μIU/ml (reference range, 0.38–4.31), that of free thyroxine (FT4) was 0.61 ng/dl (reference range, 0.82–1.63), and that of free triiodothyronine was 1.67 ng/dl (reference range, 2.17–3.34) (see Table ). An electrocardiogram showed a sinus bradycardiac rhythm (HR, 53 beats/min) with no conduction or repolarization abnormalities. The echocardiogram revealed no abnormalities. We suspected hypopituitarism based on the patient’s hypoglycemia, hypotension, nonelevated TSH, and low FT4, and also based on the results of head computed tomography (CT) and magnetic resonance imaging (MRI).
CT of the head demonstrated a high-density area in the pituitary-hypothalamic axis, which suggested a hemorrhagic lesion (Fig. ). Subsequently, MRI of the brain was performed, revealing multiple masses (Fig. a, b) and a 10-mm mass on the recessus infundibulum in T2 star-weighted sequences, which were also consistent with hemorrhagic masses (Fig. c, d). In addition, a chest x-ray revealed a left hilar mass (Fig. ). We also noted a suspicious shadow in the left lung hilum shown in the chest radiograph. Subsequently, CT of the thorax revealed a mass in the left hilum (Fig. ), which was confirmed on biopsy by bronchoscopy later. There were no emphysematous changes on the chest CT scan, but some mediastinal lymphadenopathies were detected.
After the patient’s admission, we first performed a hormone loading test on day 4. The results of the hormone profile at baseline were as follows: TSH 0.586 μIU/ml, follicle-stimulating hormone (FSH) 0.7 mIU/ml (reference range, 2–8.3), luteinizing hormone (LH) < 0.1 mIU/ml (reference range, 1.2–7.1), prolactin 37.9 ng/ml (reference range, 3.6–12.8), testosterone < 4.3 ng/dl (reference range, 142.4–923.1), adrenocorticotropic hormone (ACTH) 5.6 pg/ml (reference range, 7.2–63.3), and cortisol 0.2 μg/dl (reference range, 4.5–21.1). The results showed a TSH response to thyrotropin-releasing hormone: serum TSH 4.526 μIU/ml at 30 min and 4.591 μIU/ml at 60 min. We demonstrated an adequate cortisol and ACTH response to corticotropin-releasing hormone: serum cortisol was 5.6 μg/ dl at 60 min and had a peak of 5.7 μg/ dl at 90 min, whereas serum ACTH showed a peak of 106.3 pg/ml at 30 min, 84.0 pg/ml at 60 min, and 62.4 pg/ml at 90 min. It also showed a delayed LH and FSH response to LH-releasing hormone: serum LH showed a value of 0.8 mIU/ml at 30 min, 1.1 mIU/ml at 60 min, and peak of 1.3 mIU/ml at 90 min, whereas serum FSH was 1.5 mIU/ml at 30 min, 1.8 mIU/ml at 60 min, and peak of 2.1 mIU/ml at 90 min (Fig. a). Those results suggested hypothalamic pan-hypopituitarism.
Tumor markers demonstrated elevated pro-gastrin-releasing peptide with a value of 678 pg/ml (reference range, 0–80.0) and neuron-specific enolase 18.6 ng/ml (reference range, 0–12), which are typical markers for small cell lung cancer. We performed lung biopsy by bronchoscopy. Endobronchial ultrasound of bronchoscopy was used, and transbronchial lung biopsies were performed. The pathological results eventually revealed a small cell lung cancer (Fig. ).
We made a diagnosis of pan-hypopituitarism secondary to suprasellar metastases from a small cell lung cancer and first initiated hormone replacement therapy with hydrocortisone and levothyroxine. Right after the replacement therapy, the symptoms of lethargy, loss of appetite, and hypotension were improved. There was no evidence of diabetes insipidus during those therapies. The patient’s clinical stage was stage IV (cT3N2M1b), and his performance status was 0 to 1; thus, he was treated with adjuvant cranial radiotherapy on day 24 and chemotherapy with cisplatin and etoposide on day 45. We reevaluated the hormone loading test on day 59 after admission, which revealed an improvement in ACTH and cortisol secretions (Fig. ). We also performed a brain MRI in T2 star-weighted sequences again on day 62, which demonstrated disappearance of the suprasellar tumor (Fig. e, f). The patient was subsequently treated with four cycles of chemotherapy, but he died 10 months later due to the progression of the lung cancer. An autopsy was not performed, because the patient’s family denied permission.
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pmc-6240555-1
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A 41-year-old woman with past medical history of peripartum cardiomyopathy, mitral regurgitation, and hypertension was referred to the emergency department (ED) due to severely elevated blood pressure. Patient reports a one week history of dyspnea, mild chest pressure with exertion, and stated that she had similar symptoms before with her pulmonary embolism more than 10 years ago. Three days prior to the onset of her symptoms, the patient had stopped taking her prescribed hydrochlorothiazide (HCTZ) due to increased urinary frequency, but maintained compliance with losartan. Vital signs at presentation were temperature 37.2 °C, BP 218/150, heart rate 121, respiratory rate 16, and pulse oximetry 100% on room air. On exam, the patient was well-appearing and in no apparent distress. The patient’s lungs were clear to auscultation and she had no S3, jugular vein distention, or lower extremity edema. The remainder of the physical exam was unremarkable. Further testing included labs, an electrocardiogram (EKG), a chest radiograph, and bedside echocardiogram (BSE) performed by ultrasound trained EPs. An apical four-chamber was obtained to calculate peak longitudinal strain (PLS) using only this view. Two initial troponin levels were mildly elevated at 0.08 µg/L, but down-trended thereafter to 0.05 µg/L. There was mild cardiomegaly and increased pulmonary vasculature on chest x-ray, and a new left bundle branch block (LBBB) on EKG. At the time of the initial BSE, BP was 252/163 [mean arterial pressure (MAP) = 170)] and PLS was − 3.5% (Fig. ). The EF was not calculated, but estimated to be mildly reduced. Six hours later, the BP was 171/94 (MAP = 123) and a repeat BSE was performed and PLS was recalculated. Between the first and second BSE, the patient had received a total of 60 mg IV labetalol, 25 mg PO HCTZ, 40 mg IV furosemide, and was on a nitroglycerine drip at 40 mcg/min. The MAP had been reduced by 27% and the PLS improved to − 14% (Fig. ). Repeat EKG after the IV medications continued to show a persistent LBBB. The patient was admitted to the cardiac intensive care unit for hypertensive emergency and acute coronary syndrome rule-out. Follow-up outpatient notes indicate that the patient was discharged home the following day and did not get re-admitted to the hospital within 30 days.
Figure reflects the patient before hypertension treatment and Fig. reflects the patient after treatment. Both Figs. and are quad displays of an apical 4-chamber image demonstrating peak longitudinal strain (PLS) of the left ventricle (white arrow). In each figure, top left image (A) is a 2D depiction showing the color coding for each left ventricle (LV) segment and the PLS in the 4-chamber view; bottom left image (B) displays the peak systolic strain for each of the six LV segments in the 4-chamber view; top right image (C) displays strain (y-axis) plotted over time (x-axis) for each of the six color-coded LV segments in a linear graphical display. The white dotted line shows the average of the six strain curves; bottom right image (D) shows the anatomical M-mode display depicting instantaneous strain for the 4-chamber plane with each LV segment color-coded on the y-axis, where the red color represents more negative strain.
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pmc-6240700-1
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A 42-year old obese, smoker and schizophrenic male was medicated with 600 mg of clozapine per day. He was admitted to the emergency department with a 2 week history of diffuse abdominal pain, abdominal distention, anorexia and semi-liquid stools. On physical examination he had 110/63 mmHg of blood pressure, he was tachycardic (heart rate = 112 beats per minute), febrile (temperature = 38.5 °C) and presented abdominal tenderness and peritoneal sign.
Laboratory investigations showed a hemoglobin of 13.6 g/dl, an increase in inflammatory markers (white blood cells 13.2 × 109/l, C-reactive protein >32 mg/dl) and a renal insufficiency (creatinine 3.02 mg/dl, urea 189 mg/dl).
An upright abdominal X-ray demonstrated a pneumoperitoneum which was confirmed by the abdominal and pelvic computerized tomography ( ).
He was subjected to an emergency laparotomy where multiple punctiform perforations (holes smaller than 1 cm) in the anti-mesenteric border of the distal jejunum and ileum were identified. Purulent peritonitis was present. A small bowel resection of 1.5 m was done ( ).
On the second day of the postoperative period, an anastomosis dehiscence was registered. A subsequent re-laparotomy was needed. An anastomosis and caecum resection was done with the creation of an end-ileostomy and a colostomy. During hospitalization he had a respiratory tract infection which was treated with broad-spectrum antibiotics and an intra-abdominal abscess which was treated with percutaneous drainage. On the 28th day of hospitalization the patient was discharged.
Histologic specimens revealed non-specific inflammatory findings with ischemia.
The investigation was carried out with microbiologic cultures, serologic tests, laboratory tests, endoscopic exams with biopsies and other diagnostic exams. The main causes of spontaneous small bowel perforation were excluded, such as, infectious (cytomegalovirus, tuberculosis, bacterial, parasitic and protozoal), immune (Crohn’s disease, celiac or gluten-sensitive enteropathy and vasculitis), congenital (Meckel diverticulum and small bowel diverticulum or duplication), vascular and neoplastic.
The dose of clozapine was reduced because the suspension was not viable and a restoration of the bowel continuity was done ten months later.
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