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pmc-6240708-1
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A 50 year old man presented with complaints of suprapubic swelling and difficulty in micturition for the last 4 months. He had to strain to pass urine and the flow was poor. Patient also complained of constipation for the last 2 months. No history of fever, vomiting, hematuria and bleeding per rectum.
On examination he had pulse rate of 86/min and blood pressure – 110/76 mmHg. General physical examination was within normal limit. Abdominal examination revealed a smooth, firm, slightly tender, nonmobile lump in suprapubic region reaching approx. 5 cm above pubic symphysis, lower limit not palpable. On digital rectal examination, a smooth spherical mass was felt anteriorly and laterally outside the rectal wall, rectal mucosa was normal.
Investigations revealed haemoglobin of 12.4 g/dl, total leucocyte count of 8900/mm3, platelet count of 2.54 lakh/mm, blood urea 33 mg/dl, serum creatinine 1.12 mg/dl, and serum electrolytes were normal. Liver function test showed serum bilirubin of total – 0.75 mg/dl, S.G.O.T. – 16 U/L, S.G.P.T. – 36 U/L, and alkaline phosphatase – 88 U/L. Urine examination showed pus cells (5–6/hpf) but the urine culture was sterile. Chest X-ray and ECG were normal.
Ultrasound examination revealed a cystic mass in the pelvis suggestive of a hydatid cyst with bilateral hydroureteronephrosis more on right side as compared with left. Liver and spleen were normal.
Computerized tomographic scan was suggestive of well - defined capsulated heterogeneously within, compressing the urinary bladder and rectosigmoid and reaching till the pelvic side walls – likely hydatid cyst, moderate hydronephrosis on right side and mild hydronephrosis on left side with dilated and tortuous both ureters (a–c).
Patient was put on one cycle of preoperative albendazole therapy (10–15 mg/kg/day) for 28 days. Exploratory laparotomy was done and liver, spleen, mesentery, omentum were found to be normal. A large tense hydatid cyst was noted in the pelvic cavity, densely adhered to urinary bladder, sigmoid mesocolon, rectum and iliac vessels laterally. Upper part of cyst was separated anteriorly from the urinary bladder and on left side from sigmoid colon and mesocolon. After mobilization hydatid cyst was isolated by packing surrounding area with 0.5% cetrimide soaked sponges and cyst opened under controlled condition. All daughter cysts and laminated membrane removed completely (a, b). The part of ectocyst which was densely adherent to vital neighbouring structures could not be removed. A drain was placed in pelvis and abdomen closed in layers. Final diagnosis was confirmed by histopathological examination.
Postoperative period was uneventful and patient was discharged on 5th postop day. Patient was put on 3 cycles of albendazole therapy. Each cycle of albendazole therapy was of 28 days duration. After each cycle patient was advised a gap period of 2 weeks, and in that period liver function test and complete blood counts were assessed and found to be normal. Patient was symptom free after 6 months of follow up.
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pmc-6240723-1
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A 16 years old young man came to Geisinger Emergency Department because of right lower quadrant abdominal pain that was acute in onset and had worsened over the prior two days; it was accompanied by fever, anorexia, nausea, and vomiting.
PMH: He was an accomplished athlete; and his father’s over-riding concern was that his son be evaluated thoroughly and treated expeditiously, so as to resume football practice as soon as possible!
Two years ago he had fractured his right clavicle; and because of the poor alignment and delayed healing, operative reduction and fixation was performed ().
He was remarkably tender in the right lower quadrant of his abdomen, with involuntary guarding and positive psoas and obturator signs. His WBC count and Urinalysis were normal. Ultrasound identified “mildly prominent, non-specific lymph nodes” but not the appendix. The MRI was also “somewhat equivocal” -free fluid in the pelvis with inflammation, but the appendix was not visualized; nevertheless, the radiologist opined that the findings were consistent with either appendicitis or inflammatory bowel disease. Unfortunately, the MRI did not include the pubic symphysis and contiguous musculature.
The diagnostic impression was appendicitis; however, this was not corroborated by laparoscopy, which revealed a normal appendix. No other intra-abdominal pathology was identified. The presumptive diagnosis became gastroenteritis, and an uneventful recovery was anticipated. This prediction initially appeared correct, and he was discharged only to return the evening of the second post-operative day, complaining once again of exquisite right lower quadrant abdominal pain, this time associated with fever, leukocytosis, and elevated inflammatory markers ().
The second admission’s CT and MRI demonstrate (, ):Fluid in the retro-pubic space of Retzius Two rim enhancing collections within the pectineus and rectus abdominal muscle denoting either myositis or a periosteal abscess Blurring of the cortex (growth plate) of the right superior pubic ramus
Blood cultures were obtained, and he was treated empirically with NSAID’s and antibiotics to cover presumed osteitis pubis or osteomyelitis. Clindamycin was switched to Cefazolin, because the blood culture grew MSSA, resistant to Clindamycin. Clinical improvement was followed by discomfort in his right shoulder, and radiographs disclosed septic arthritis in the sternoclavicular joint ().
The consultant orthopedist explored the sternoclavicular joint and drained approximately 10 ml of purulent material. The joint was irrigated; infected bone and cartilage were curetted; and adjacent soft tissues were debrided. The hardware was not removed, but ultimately this might be necessary.
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pmc-6240724-1
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A 22-year old woman presented to our clinic with a palpable mass for 6 months. The mass was painless. Her medical history was not remarkable for any disorder. On physical examination she had a palpable mass filling the left upper quadrant and epigastrium. On laboratory examination she had normal levels of total protein, albumin, globulin, alanine aminotransferase, aspartate aminotransferase, blood urea nitrogen, serum creatinine, carbohydrate antigen 19–9 (Ca19-9), carcinoembryonic antigen (CEA) and alpha-fetoprotein (AFP). She also had negative serology for hepatitis B and C viruses. On ultrasonography there was a hypoechoic, solid mass with sharp contours and heterogenous pattern which had a size of 16 x 10 cm and diffuse cystic-degenerative areas and which appears hypervascular on Doppler USG (A). The described mass was considered to reside exophytically in the left lobe of the liver. An urgent abdominal tomography showed a giant solid mass that originated from the inferior part of the medial segment of the left lobe of liver and that extended inferiorly. Its size was approximately 17 × 15 x 11 cm. It had smooth contours and marked hypervascularity. It contained diffuse cystic-degenerative areas. A giant hepatic adenoma was primarily considered in the differential diagnosis, which also included liver tumors of mesenchymal origin or hepatocellular carcinoma on a non-cirrhotic basis (B).
The patient’s abdominal cavity was explored with a subcostal incision. There was a mass with smooth contours, measuring 15 x 12 cm in the left lobe of the liver, which grew exophytically. Other parts of the liver were normal. The mass’s portion out of the liver was of hypervascular appearance that compressed adjacent tissues but was easily separable from them. The mass was excised with liver tissue and gall bladder, with a negative surgical margin, with the help of an ultrasonic dissector and cautery. There was no additional lesion in the abdominal cavity ().
The macroscopic examination of the hepatic resection material revealed a tumoral lesion with a size of 14 × 12 x 13 cm and a cross-sectional color of yellow, which contained diffuse hemorrhagic and necrotic areas, 2 cm apart from the surgical margin. Sections prepared from the tumor showed that it was separated from the adjacent hepatic parenchyma with a clear border but showed infiltration of the parenchyma in a few foci (A). The tumor was highly cellular, the components of which were spindle in shape from place to place and epithelioid in most areas, and they had round-ovoid nuclei and abundant eosinophilic cytoplasm (B). There were interspersed cells that showed nuclear coarsening. Tumor’s background was highly rich in vascularity and there were interspersed free hemorrhagic foci.
Immunohistochemical study showed negative staining with Pan-CK, Hep-Par, CD117. There was diffuse cytoplasmic positivity with HMB-45 (C) and SMA (D). The background rich vascular network was positively stained with CD34, CD31 and Factor 8 while tumor cells were not. Two mitotic figures were noted under 50 gross magnification. Morphological appearance and immunohistochemical study results suggested a PEComa. Although the criteria for malignancy have not been clearly defined for hepatic PEComas, considering a tumor size greater than 5 cm, presence of more than 1 mitosis under 50 GMA, and infiltrative growth pattern, which have been associated with tumor recurrence or metastatic process for soft tissue or gynecological tumors, the case was considered a malignant PEComa.
The patient was discussed in general surgery and oncology councils, which recommend no therapy. The patient recovered uneventfully, and no additional therapy was recommended. She was discharged 3 days after the surgery. She was put under close follow-up; her tri-monthly control tomographic examinations revealed no pathology. She is under follow-up without recurrence 10 months after the surgery ().
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pmc-6240725-1
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A ten-year-old girl was admitted to our general hospital with numbness of her left palm and fingers in the last 5 months before admission. At that time, she was hit by a car while she was riding a bicycle. The car was coming from opposite side, and she fell with her left forearm was sliced by licensed plate of the car. There was a semicircular open wound with active bleeding on the left forearm, and she was in pain. She was brought to a nearby clinic and had her left forearm sutured. After the pain subsided, she felt numbness of her left hand and fingers. In addition, she could not extend her fingers. Finally, the patient decided to seek medical attention and get further treatment at our general hospital.
From physical examination, there were claw hand deformity with thenar and hypothenar atrophy as well as a scar on the anterior side of distal forearm (). Sensorium loss of the palm and third, fourth, and fifth fingers was impaired. No tenderness was found. Capillary refill of the fingers was normal. Range of motion of the fingers was altered with limitation of finger abduction and thumb apposition (). Moreover, range of motion of the wrist was within normal limit.
Routine laboratory examination was within normal limit. The patient was taken for wrist and forearm radiographs and, similarly, there was no abnormality depicted on either bones or soft tissue.
The patient also underwent electromyography examination which showed median and ulnar nerve lesion at the left forearm with total axonal degeneration. No signs of reinnervation of both peripheral nerves were detected.
The patient was diagnosed as ulnar and median nerve palsy of left forearm, and then we planned to perform surgical exploration of the nerves and to repair with sural nerve graft, Zancolli procedure and sural nerve graft.
Intraoperatively, skin incision was made on the previous surgical scar. Injury site was explored, and complete rupture of both ulnar and median nerves was found. Degeneration of both nerves was also seen, with neuroma rising from both the proximal stumps. The proximal and distal ends of both ulnar and median nerves was cut until nerve fascicle was visible. The distance between proximal and distal stump was measured: for ulnar nerve the distance was 7 cm, while it was 8 cm for median nerve. Sixteen centimeters of ipsilateral sural nerve was harvested, and the ulnar and median nerves were repaired using the nerve graft. Then Zancolli procedure was performed: skin incision was made along the palmar crease, A1 pulley was identified around metacarpophalangeal joint, longitudinal incision was made on the pulley, flexor digitorum superficial tendon was retracted laterally, metacarpophalangeal joint capsule was identified, an elliptical incision was made over the joint capsule, and capsulodesis was performed. Postoperatively the wound was closed and immobilized by elastic bandage ().
We followed the patient at 3-week postoperatively, and the patient had improvement of her claw hand (). She was advised to continue her rehabilitation of her hand to further improve her hand function, especially opposition and key pinch. At 6-month follow-up, she had improved grip strength and normal functional level of her left hand. At 2-year follow-up, she could handle daily activity as before the accident and was satisfactory with her condition. ()
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pmc-6240735-1
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We investigated the molecular basis of a presumed mitochondrial disorder in a 10-year-old British female of mixed ethnic background, previously reported in the literature (). She is the third child of a healthy, non-consanguineous parents, with no familial history of neurological disease apart from epilepsy in a maternal aunt. Intrauterine growth retardation was observed at 32 weeks of pregnancy. At birth, she had a low birth weight (2.33 kg) and below-average height (45 cm) and showed reduced spontaneous movements and hypotonia. The subject presented with feeding difficulties, gastroesophageal reflux with projectile vomiting and acetabular dysplasia. Brain magnetic resonance imaging at 8 months revealed a delay in white matter myelination. She remained proportionately small (height, weight and head circumference all below the 0.4th centile) and weak throughout childhood. Biochemical analyses revealed increased lactate (4.2 mmol/L; normal controls, 0.5–2.2 mmol/L) and creatine kinase (282 U/L; normal controls, 100–190 U/L) levels in plasma and she underwent a diagnostic muscle biopsy on suspicion of mitochondrial disease. This identified decreased activities of respiratory chain complexes I, III and IV with sparing of complex II activity (). Having excluded mitochondrial DNA (mtDNA) rearrangements and a quantitative loss of mtDNA copy number, full mtDNA sequencing revealed a rare homoplasmic m.5514A>G mt-tRNATrp variant not present in >3000 human mtDNA control sequences (). The m.5514A>G transition affects an A-U base pair in the acceptor stem of mt-tRNATrp, however, this position in the transfer RNA
(tRNA) molecule shows poor evolutionary conservation (). The m.5514A>G variant was homoplasmic in the blood from her clinically unaffected mother, prompting further studies to assess pathogenicity and the possible implications of this rare mtDNA variant at a cellular level.
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pmc-6240820-1
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A 56-year-old woman was referred to our outpatient clinic in 2018 because of pain and right hip decreased range of motion. She underwent a right-sided THA in 2001, when a modular neck implant and femoral stem with proximal titanium porous coating were used (acetabular cup: SPH-CONTACT; femoral stem: F2L Multineck; Lima Corporate, Villanova San Daniele del Friuli, Italy). Early postoperative period was uneventful. In 2012, the patient sustained right-sided trans-acetabular and inferior pubic ramus fractures, which were successfully treated conservatively. Since 2016 she complained about increasing pain in the right groin region and had severely reduced right hip range of motion. Examination in our outpatient clinic showed that her right leg was 2 cm shorter.
The initial x-ray examination in 2018 showed acetabular cup dislocation, eccentric femoral head wear, “cloudy bubbles” characteristic of metallosis, and pseudotumor formation (). It also showed damage to the titanium porous coating of the femoral stem. A review of the medical records from 2016 revealed femoral head wear in situ and damage to the porous stem coating. A revision surgery was indicated, and the patient agreed to the procedure.
During surgery, performed using direct lateral approach, extensive metallosis was observed (, Supplementary Video 1). After thorough debridement and irrigation, all implant components were removed (). The femoral head was gravely worn and elliptically shaped (). The polyethylene liner on the acetabular side had no visible holes or cracks, suggesting there was no direct contact between the femoral head and metal acetabular shell. After endoprosthesis extraction, notable polyethylene liner wear was visible, with metal debris covering the inner surface (). Due to a large acetabular bone defect, it was decided not to proceed with a new acetabular cup implantation. In the postoperative period, a coxofemoral orthosis was applied, and crutches were used for touchdown weight-bearing only. Intraoperatively collected microbiology culture swabs were negative for aerobic and anaerobic microorganisms.
Macro- and micro-structure of all extracted components were analyzed in detail at the Faculty of Mechanical Engineering and Naval Architecture of the University of Zagreb. The analysis revealed macroscopic damage to the titanium porous coating of the femoral stem () and a decreased volume of the femoral head. Metal debris on the acetabular liner was distributed heterogeneously; fewer debris particles were present on the part of the liner adjoining the worn femoral head than on the remaining part of the liner. The surface damage features of the polyethylene were characterized by scanning electron microscopy (SEM) (TESCAN VEGA TS5136LS, TESCAN ORSAY HOLDING a.s., Brno-Kohoutovice, Czech Republic). SEM revealed damage, scratches, indentations, and embedded metal debris particles on the whole acetabular liner surface (). Chemical composition and origin of these particles were determined using energy-dispersive spectroscopy (EDS, OXFORD Instruments, Abingdon, UK), which showed that metal debris consisted of both titanium and stainless-steel particles ().
Femoral head component (AISI 316L stainless-steel) was removed from the femoral neck, and no signs of trunnionosis were observed at the head/neck junction (). To determine the wear mechanisms, the wear tracks on the femoral head surface were analyzed by SEM, and only traces of abrasive wear were found (). The microstructure of the femoral head material was typical for this type of material: homogeneous austenitic without any significant defects or other irregularities (), with microhardness of 145 HV 0.2 (mean value of 5 measurements).
The patient signed the informed consent for publishing the medical data and visual materials.
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pmc-6240939-1
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A 37-year-old man was admitted to hospital for several months of headache, hoarseness and dysphagia; a month of right-sided deafness and nasal bleeding; and a week of dysarthria. He had experienced sinusitis for 1 year before admission and had been treated with antibiotics. He was successfully treated with glucocorticoids (GC) for sudden right-sided hearing loss 9 months before admission. His body weight had decreased by 10 kg over the previous month. A week before admission, he developed a right steppage gait and numbness in the right L5 distribution.
On admission, body temperature was 37.7 °C and the rest of his vital signs were normal. Neurological examination showed a bilateral mixed hearing loss, a right curtain sign, weakness of the right trapezius, rightward tongue deviation, and paralysis of the right peroneal nerve. Initial blood tests showed a slightly elevated erythrocyte sedimentation rate (29 mm/h) and C-reactive protein (CRP) levels (1.06 mg/dL), and white blood cell count was slightly increased (8.9 × 109/L). His renal and liver function was normal (eGFR 118 ml/minute/1.73 m2) and the urine test was also normal (proteinuria, hematuria, urinary cast were negative). Anti-nuclear antibody, rheumatoid factor, angiotensin converting enzyme, myeloperoxidase-anti-neutrophil cytoplasmic antibody and soluble interleukin-2 receptor were normal, but proteinase 3-anti-neutrophil cytoplasmic antibody was increased (16.9 IU/mL). Cerebrospinal fluid was normal. A gadolinium-enhanced MRI scan of the head showed an enhancing infiltrative lesion in the right retropharynx encasing the carotid sheath (Fig. ), which seemed to cause the paralysis of IX, X, XI and XII nerves. Lumber spine MRI showed no evidence of lumbar disk herniation and nerve conduction study showed the paralysis of the right peroneal nerve. Chest computed tomography showed a 23 mm nodule in the left upper lobe. A CT-guided needle biopsy of the lung lesion and biopsy of the nasal mucosa were performed but showed only infiltration of inflammatory cells and no evidence of malignancy, vasculitis or granuloma. Culture of the lung specimen showed no evidence of infection and the interferon-gamma release assay for Mycobacterium tuberculosis was negative. We diagnosed GPA based on the American College of Rheumatology classification criteria for Wegener’s granulomatosis (WG) and classification of WG by the Watts algorism [, ].
The patient was treated with prednisone (PSL) 1 mg/kg daily and IVCY 1000 mg every 3 weeks (Fig. ). His fever, headache, and swallowing function improved rapidly, and peripheral neuropathy also improved, but hoarseness persisted. His Birmingham Vasculitis Activity Score 2008 version 3 score improved from 14 to 4, and he was discharged on IVCY and a tapering dose of PSL 45 mg/day [].
Three months later, while receiving oral GC and IVCY, he was readmitted for recurrence of headache and right-sided hearing loss. On MRI, the lesion encasing the right carotid sheath has not reduced (Fig. ). CRP levels remained slightly increased (1.04 mg/dL). He was treated with RTX 600 mg/week for 4 weeks and GC for re-induction therapy. His headache improved rapidly and hearing ability improved slowly. He was still on GC 1 year after RTX administration, with persistent hoarseness as his only symptom.
To investigate immune status, we tracked peripheral T and B cell counts after RTX administration (Fig. ). According to the peripheral T and B cell counts, whereas CY treatment with GC reduced the number of peripheral CD4+ and CD8+ T cells and B cells, RTX selectively reduced the number of peripheral B cells slowly over time. B cells tended to decrease 2 weeks after RTX treatment, with the reduction lasting 8 months.
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pmc-6240944-1
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A right-handed 66-year-old Japanese man experienced right elbow pain and was unable to extend his right thumb and fingers for 1 month. He did not have associated history of trauma to his elbow or any remarkable medical history. A physical examination showed swelling of his right elbow and a palpable mass on the anterior aspect of his right elbow. Grip strengths of his right and left hands, as measured with a Jamar digital dynamometer (Takei Scientific Instruments Co., Ltd., Niigata, Japan), were 30.4 and 35.0 kg, respectively. The respective ranges of motion for his right and left extremities, as measured with a standard goniometer, were as follows: elbow flexion, 115° and 145°; elbow extension, − 15° and 0°; forearm pronation, 30° and 70°; forearm supination, 80° and 90°. The muscle strengths of his right upper extremity, as evaluated using the British Medical Research Council scale, were the following: triceps, 5; wrist extensor, 5; extensor pollicis longus, 3; extensor digitorum communis and extensor indicis proprius (EIP), 2. There was no sensory loss.
Plain radiographs of his right elbow showed osteoarthritic change with calcifications and ossicles anteriorly, posteriorly, and laterally (Fig. a, b). Plain computed tomography (CT) and three-dimensional CT scans (Activion 16; Toshiba Medical Systems Corp., Tokyo, Japan) showed clustered calcifications around the radial neck, coronoid fossa, radial fossa, and olecranon fossa. Plain magnetic resonance imaging (MRI) scans (EXCELART Vantage 1.5 Tesla, version 9.51; Toshiba Medical Systems Corp.) showed mass lesions around the radial neck, medial epicondyle, olecranon fossa, and coronoid fossa, with heterogeneous intensity on T1-weighted and T2-weighted images (Fig. c, d). A nerve conduction study (NCS) for his radial nerve was performed. For the motor NCS, which was recorded at the EIP, surface electrodes for stimulation were set at 8 cm proximal to the EIP, 5 cm proximal to the elbow crease, and posterior to the insertion of the deltoid. For the antidromic sensory NCS, it was recorded at the middle between the first and second metacarpal bones; surface electrode for stimulation was set at 14 cm proximal to the recording position. The respective right and left motor nerve conduction velocities were 70.2 and 57.7 m/s from the upper arm to the elbow and 29.9 and 66.1 m/s from the elbow to the forearm. The results of sensory nerve conduction velocities were 62.5 and 57.9 m/s, respectively.
The quality of the three-dimensional CT images was poor for detecting the tumor so we created three-dimensional reconstructed images of the tumor based on MRI to visualize the shape and location of the tumor and its relation to the radial nerve. Digital imaging and communications in medicine data obtained from plain magnetic resonance (MR) images were transferred to Mimics computer-aided design (CAD) software (Materialise Japan, Yokohama, Japan). The bone region was segmented semi-automatically with an intensity threshold segmentation technique, and the tumor and nerve (radial nerve, PIN, and superficial branch of radial nerve) intensity was contoured manually using the CAD software; then, three-dimensional reconstruction was performed (Fig. a–c). The three-dimensional images clearly showed the tumor location and morphology; we found a giant tumor around the radial head and neck and a large mass in the radial fossa, olecranon fossa, and medial to the coronoid fossa. Moreover, the running course of the PIN was extremely changed at the distal corner of the tumor in contrast to the superficial branch of the radial nerve.
We diagnosed our patient as having incomplete PIN palsy caused by synovial osteochondromatosis. We decided to resect the tumor from the anterolateral part and radial fossa and perform neurolysis of the PIN because our patient had no impairment in daily life, for example, limitations in range of motion were compensated by shoulder joint motion.
Surgical treatment was performed via the anterolateral (Henry) approach using an air tourniquet, with our patient under general anesthesia. The three-dimensional reconstruction images were used as a basis for the surgical exposure, and the PIN was compressed between the arcade of Frohse and the tumor, which was under the PIN (Fig. a). The PIN was kinked, especially at the corner of the tumor, as shown by the three-dimensional reconstruction image. After the incision of the arcade of Frohse was made, neurolysis of the PIN was performed. The tumor was covered by the joint capsule (Fig. b). When the joint capsule was incised, a white, cartilaginous tumor was found. The tumor, including the synovium, was resected (Fig. c). Results of a histological analysis showed synovial osteochondromatosis without malignancy. No major postoperative complications occurred. Our patient fully recovered from the PIN palsy 9 months postoperatively. One year postoperatively, his grip strengths were 36.7 and 33.7 kg for his right and left hands, respectively. The ranges of motion of his right extremity were as follows: elbow flexion, 125°; elbow extension, − 15°; forearm pronation, 65°; forearm supination, 90°. No recurrence of the lesion occurred 1 year postoperatively.
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pmc-6240963-1
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On 26th May, 2017, a 49 years old male patient, from Ambiri Village demonstrated predominant gastrointestinal symptoms of nausea and diarrhoeas accompanied by fever. He was treated at home by paramedics but his condition deteriorated and he died suddenly after experiencing heavy diarrhoeal episodes. Since he was not taken to a hospital, further investigations could not be done. According to his family he has been involved in the slaughter of a cow before the onset of symptoms.
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pmc-6240963-2
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The fifth case was a 53 years old male and had been involved in butchering and skinning of the same cow, as the other patients. He developed similar gastrointestinal symptoms as well as additional haemorrhagic signs (melena) and also was admitted to Lady Reading Hospital Peshawar. His blood specimen was sent to the hospitals laboratory for routine investigations and additionally to the National Institute of Health (NIH) for detection of the Dengue NS-1 antigen, CCHF virus antigen and genomic RNA, using a real-time reverse transcriptase polymerase chain reaction (RT-PCR) (Fig. ) assay []. The CCHF-PCR showed a positive result confirming the suspected infection. The patient was treated with ribavirin and supportive treatment and survived the infection.
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pmc-6241070-1
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A 13-year-old Japanese girl was admitted to our hospital due to fever, fatigue and abnormal blood test results. She had a history of chronic constipation and repeated (once or twice a year) intermittent hepatic impairment associated with acute febrile illnesses, but she had not received any detailed examination for these symptoms. Her latest medical record showed that she presented to her family doctor 15 months prior to admission to our hospital, following a febrile episode. Her laboratory results for aspartate transaminase (AST), alanine transaminase (ALT), total bilirubin (T-Bil), and alkaline phosphatase (ALP) showed levels of 406 IU/L (reference range 14–29 IU/L), 227 IU/L (reference range 9–28 IU/L), 0.5 mg/dL (reference range 0.25–1.20 mg/dL), and 798 IU/L (reference range 220–1250 IU/L), respectively. Her hepatic impairment resolved spontaneously after the fever subsided. Other medical and family histories were unremarkable. The day before her admission, she had visited her family doctor complaining of fever for 2 days and weariness. On the following day she remained febrile and vomited three times; following this, she consulted the emergency pediatric service at night, where blood analysis was performed. It revealed abnormal liver function test results, with AST levels of 681 IU/L and ALT levels of 547 IU/L; therefore, she was referred to our hospital for a thorough investigation. On admission, she had an axillary temperature of 37.9 °C and tachypnea of 30 per minute. Other physical examination findings were normal. Although she had no apparent respiratory symptoms other than tachypnea, we performed a chest X-ray and mycoplasma loop-mediated isothermal amplification (LAMP) assay because Mycoplasma pneumoniae pneumonia was locally prevalent. Chest X-ray revealed slight bilateral reticular opacity, which is a clinical feature suggestive of viral or atypical pneumonia.
Following a positive result in the LAMP assay, we diagnosed her with Mycoplasma pneumoniae pneumonia, and oral azithromycin was initiated. The fever and weariness of the patient completely subsided with medication and resting; she was discharged after 4 days of admission.
To elucidate the etiology of her hepatic impairment, we performed blood analysis on admission, which revealed AST levels of 394 IU/L, ALT levels of 401 IU/L, T-Bil levels of 0.77 mg/dL, ALP levels of 885 IU/L, and gamma glutamyl transferase (γ-GTP) levels of 146 IU/L (reference range 8–36 IU/L) (Table ).
Complete blood cell counts, coagulation test results, antinuclear antibody titer, and viral tests for hepatitis viruses, Epstein-Barr virus and cytomegalovirus were all normal. Serum levels of copper, ceruloplasmin, and alpha-fetoprotein are unremarkable.
With respect to imaging studies, initial abdominal ultrasonography revealed a normal liver; however, the gallbladder could not be visualized. A second abdominal ultrasonography was performed following fasting; however, visualization of the gallbladder was impossible. MRCP was carried out on the fourth day of admission, which revealed no major abnormality except for the absence of the gallbladder (Fig. ).
We performed further studies on an outpatient basis, which revealed no anomaly other than the absence of the gallbladder. Six weeks after discharge, her laboratory tests showed normal levels of AST, 29 IU/L; ALT, 9 IU/L; T-Bil, 0.90 mg/dL; ALP, 415 IU/L; and γ-GTP, 13 IU/L (Table ). To confirm the absence of the gallbladder, we performed drip infusion cholecystocholangiography with computed tomography (DIC–CT); this imaging study uses a radiopaque dye that preferentially accumulates in the tissue of the gallbladder or bile ducts. The DIC-CT study could not reveal the gallbladder in the usual nor aberrant position (Fig. ).
Based on these results, the patient was diagnosed with gallbladder agenesis. During outpatient follow-up, she gradually manifests nausea after eating fatty foods; therefore, we prescribed smooth muscle relaxant and ursodeoxycholic acid. The outpatient follow-up is still ongoing, and febrile episodes have not been observed after discharge.
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pmc-6241237-1
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A 74-year-old female patient was referred to the Tartu University Clinic with impaired consciousness and tetraparesis from a local hospital where she had been diagnosed with an ischaemic stroke. A month before her presentation to our clinic, the patient's symptoms had appeared as only clumsiness and a slight impairment of the sensations in her left hand. At home, her clinical state progressively worsened, and she developed difficulty with speech, walking, and swallowing. Mental deterioration of the patient was not reported, possibly due to her rapid impairment of consciousness. The patient's medical history included a myocardial infarction in 2008, hypothyreosis, increased blood pressure, and tension headaches. However, the patient was completely independent and lived with her husband.
Upon arrival to the Tartu University Clinic, the patient exhibited disturbed consciousness with a Glasgow Coma Scale (GCS) score of 9 points. Laboratory tests revealed leucocytosis and increased C-reactive protein levels. She was diagnosed with and treated for right-sided pneumonia. T1, T2, FLAIR with gadolinium contrast medium and diffusion-weighted brain MRI was performed, showing only small periventricular and subcortical white matter lesions compatible with ischaemic leukoencephalopathy. The patient was not diagnosed with any infectious, inflammatory, or neoplastic aetiology. Lumbar puncture revealed an absence of white blood cells and a protein concentration of 0.28 g/L.
On the day of admission, a 20-minute standard EEG was obtained. During the examination, the patient's GCS score was 9 points, and she exhibited occasional, subtle clonic jerks in her left arm. The EEG showed pseudoperiodic lateralized epileptiform discharges (PLEDs) over the right hemisphere at a frequency of 2–3 Hz (), which were time-locked with the clinical motor signs (video ). Surface voltage mapping showed a dipole configuration with maximal negativity over the right frontoparietal region. The nociceptive stimulation was performed, without affecting PLEDs.
After the administration of intravenous diazepam (5 mg), the epileptiform activity and left arm jerks were completely resolved, and the EEG exhibited diffuse delta activity with a frequency of 1.5 Hz. However, the patient's mental state did not improve. Based on these findings, the patient was diagnosed with possible NCSE, and antiepileptic treatment with carbamazepine and intravenous valproate was initiated. The patient did not respond with clinical improvement, despite therapeutic plasma levels of the anticonvulsive medications and the introduction of propofol anaesthesia in the intensive care unit.
A brain biopsy was performed on the right parietal lobe, revealing pathological results compatible with the diagnosis of sporadic CJD. The CSF of the patient was positive for the 14-3-3 brain protein. The patient had an intracerebral haemorrhage in the right hemisphere as a complication of the brain biopsy, and the haematoma was surgically removed. shows EEG results performed 25 days after the first EEG. The exam revealed a severe slowing of the background activity and periodic sharp-wave complexes (PSWCs) with triphasic morphology at a rate of 0.5 Hz. She was stable after the operation but died 34 days after admission. The patient's total disease duration was 60 days.
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pmc-6241238-1
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The patient is a 50-year-old man with history of myelodysplastic syndrome, which had progressed to acute myeloid leukemia. He underwent nonmyeloablative allogeneic hematopoietic stem cell transplant (HSCT) in 2013 and had numerous complications. He had graft failure and underwent a successful second allogeneic sibling peripheral blood stem cell transplant in 2014. His disease course was complicated by chronic GVHD (cGVHD) involving the eyes, skin, liver, and buccal mucosa.
For his cGVHD, he had been treated with prednisone on a taper, sirolimus, and twice weekly extracorporeal photopheresis (ECP). However, he developed a systemic infection with Mycobacterium abscessus (M. abscessus) and the ECP Vortex Port® had to be removed. As M. abscessus requires prolonged antibiotic therapy, we elected not to replace the port and he was switched to ruxolitinib, used in combination with sirolimus and prednisone. He was seen in clinic for evaluation of severe fatigue, aching abdominal pain localized to the upper abdomen radiating into the back, headache, and nausea. He noted diaphoresis, but no fevers. Initial lab work-up was notable only for albumin 2.4, alkaline phosphatase 100, ALT 105, AST 105, WBC 4.6, hemoglobin 14.2, and platelets 202. Blood sample was lipemic and triglycerides were >4000 (ref. range <150 mg/dL). Subsequent labs included amylase 12 (ref. range 30-110 U/L) and lipase 225 (ref. range 73-393 U/L). CT of the abdomen and pelvis with intravenous contrast showed no convincing evidence of pancreatitis, only a small calcific focus at the tail of the pancreas, representing either a parenchymal calcification or a small ductal stone. He was admitted to the hospital for further work-up and management.
On admission, medications included acyclovir, amlodipine, ascorbic acid, calcium carbonate, cefoxitin, docusate, fluconazole, furosemide, gabapentin, lisinopril, methadone, metoprolol tartrate, multivitamin, omega 3 fatty acids, pantoprazole, pravastatin, prednisone, ruxolitinib, sirolimus, sulfamethoxazole-trimethoprim, tamsulosin, tigecycline, trazodone, venlafaxine, cholecalciferol, and zinc acetate.
While hospitalized, sirolimus and lisinopril were held and endocrinology was consulted for evaluation of hypertriglyceridemia. Prior triglyceride levels were noted to be between 300 and 400 over the last several years. He was started on an insulin drip with a goal to reduce triglyceride level to less than 500 mg/dL. The patient's abdominal pain improved slightly. Triglyceride levels trended down from 2983 (on hospital arrival) to 2228 (on hospital day 1 after insulin) and 2093 (on hospital day 2) (see ). Therapeutic plasma exchange (TPE) was then planned to bring down the triglyceride level quickly and prevent any further sequelae of marked hypertriglyceridemia. Ruxolitinib was also stopped at that time. TPE was performed on hospital day 3 and subsequently triglyceride level decreased to 1305 and 407 on hospital day 4. He noted resolution of his abdominal discomfort with improved triglyceride levels.
The patient was instructed to follow a low-fat and low-carbohydrate diet after hospital discharge. Triglyceride levels remained high (446-881 mg/dL) for the week following discharge, but trended down to the 200s within two weeks of discharge (211-277 mg/dL) and remained within normal or minimally elevated ranges (94-270 mg/dL) for the following year. After stopping ruxolitinib, the patient's GVHD was managed with prednisone, ibrutinib, topical tacrolimus, dexamethasone oral solution, pancreatic enzymes, and artificial tears.
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pmc-6241351-1
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A 61-year-old otherwise healthy Caucasian male presented to our institution with nonspecific, flu-like symptoms. The patient had been traveling with his wife to Austria, Switzerland, and Germany the month prior to presentation. They had taken a riverboat, had been in very close proximity to other people on the cruise, and there were multiple individuals who reportedly experienced similar symptoms. Upon return to the U.S., the patient's symptoms had initially improved. However, they traveled to Creede, Colorado, where his symptoms then progressed approximately 4–16 days after potential exposure.
Initially in the emergency department, the patient complained of malaise, dyspnea, chills, mild intermittent headache, and a fever. He quickly decompensated with high fevers, tachycardia, leukopenia, and a lactic acid of 7.69. Infectious etiology was considered likely. The patient was placed on broad-spectrum antibiotics; vancomycin, piperacillin/tazobactam, and levofloxacin, and given aggressive fluid resuscitation. He was admitted to the intensive care unit and within hours deteriorated further, requiring intubation, three vasopressors, and continuous renal replacement therapy. Within twelve hours, blood cultures returned positive for gram-negative diplococci, later identified as N. meningitidis W135 serotype. Over the subsequent hospital days, the patient went into disseminated intravascular coagulopathy (DIC) and progressed to purpura fulminans (PF). His clinical status did improve over the coming days and would eventually require bilateral transmetatarsal and digit amputations, as well as allograft over these areas.
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pmc-6241371-1
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This is the case of a 48-year-old G2P2002 who originally presented to our office in March 2016 with vaginal discharge, dysuria, and dyspareunia. She had previously been evaluated by her primary care physician (PCP) where a small cyst on the anterior vaginal wall was drained. She had received antibiotic treatment without relief of her symptoms. Her past medical history was significant for multiple sclerosis, Crohn's disease, and anxiety/depression. Her surgical history was significant for cesarean section × 2, bilateral tubal ligation, polypropylene midurethral sling procedure, and cholecystectomy. On physical examination, she was noted to have a small anterior vaginal wall fold near the urethra. MRI showed a cystic mass posterolateral to the urethra measuring 2.1 × 1.7 × 2.3 cm likely representing a urethral diverticulum. The mass had signs of infection/inflammation (Figures and ). Of note, the patient also had a long history of abnormal uterine bleeding and was found to have fibroids on MRI. She underwent hysterectomy with concurrent repair of the urethral diverticulum.
In June 2016, she underwent total abdominal hysterectomy, bilateral salpingectomy, and adhesiolysis. After the hysterectomy was complete, excision of her previous midurethral sling was also performed due to the patient's chronic groin pain and recurrent UTIs. Approximately 2 cm of the polypropylene mesh was freed from the periurethral space and partially excised to the inferior pubic rami bilaterally. At the time of excision, no diverticulum was able to be identified and only an area of inflammation was visualized. A 20 gauge spinal needle was passed through that area in an attempt to aspirate fluid, but no fluid was able to be retrieved. Cystoscopy was also performed and no ostia or communication suggestive of a diverticulum was visualized. Postoperatively, patient had no complications. Although the diverticulum was not able to be isolated and repaired, the patient initially had improvement of her symptoms.
In January 2017, patient returned with vaginal discharge, status post “vaginal cyst drainage” again with her PCP, along with antibiotic therapy. MRI was repeated and significant for persistence of the cystic mass/diverticulum noted on prior MRI. Her symptoms however improved after antibiotic therapy. Further treatment was subsequently delayed due to left breast discharge and subsequent diagnosis of a breast mass.
In October 2017, patient returned with 2-week history of dysuria along with persistent discharge and urinary dribbling. MRI was repeated and significant for slight increase in size of the urethral diverticulum. The case was discussed with our staff in interventional radiology. They offered needle localization of the diverticulum prior to attempting repeat excision.
In January 2018, the patient underwent the following procedure: transvaginal ultrasound was used to identify a “thick walled diverticulum” and a 5 French catheter needle was placed into this cavity. A small wire was then passed through the catheter and into the diverticulum for localization. The catheter was removed and the wire was then secured to the patient's thigh. A u-shaped incision was then made, proximal to the urethra. After the vaginal epithelium was dissected away, the diverticulum was easily identified using the guide wire (). The diverticulum was incised longitudinally and purulent material was subsequently drained. The diverticulum was dissected laterally and anteriorly away from the urethra and subsequently removed. illustrates the size of the diverticulum. After the diverticulum was removed, there was an approximately 1 cm defect in the urethra due to a previous communication between the two areas. This was repaired with 3-0 vicryl; however, due to the chronic inflammation, the tissue was weak and a leak was noted after repair, evident after methylene blue dye injection. Therefore, the defect was reopened and reapproximated with 3-0 vicryl. This time, cadaveric pericardium was anchored over the first layer of repair. The cadaveric pericardium was soaked in normal saline for rehydration per package insert instructions. The vaginal epithelium was also mobilized and placed over the cadaveric tissue for a 3rd layer of repair to ensure adequate closure. The U-shaped incision was then closed and Premarin cream was placed over the suture line. A new foley was also placed at the end of the case. Of note, culture from the fluid expressed from the diverticulum at time of repair was negative for growth.
At her 5-week postop visit, a fluorocystogram was performed and was normal. The foley catheter was subsequently removed. At 3 and 6 months after surgery, the patient was completely asymptomatic and denied leakage of urine, urinary incontinence, dysuria, or discharge.
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pmc-6242806-1
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A 30-year-old man underwent right hepatectomy at previous hospital, due to trauma, in August 2012. At that time, a thoracotomy in the eighth intercostal space was performed for direct closure of the diaphragm. Pooling of right pleural effusion was treated with drainage up to postoperative day (POD) 9. The patient recovered and was discharged on POD 14. In November 2012, he presented with a right DH and strangulation of the ileus of the small intestine. He underwent laparotomy at the same previous hospital, found the defect which was separated from the previously sutured area of the diaphragm, and repaired it.
In May 2017, the patient presented to the local hospital with abdominal pain and vomiting and was diagnosed with an intestinal obstruction; an ileus tube was inserted and the patient was referred to our hospital for treatment. Upon admission, the physical examination was unremarkable, and laboratory findings, including complete blood count, erythrocyte sedimentation rate, and biochemical tests, were all within normal limits. Chest and abdominal radiographs revealed colon gas in the right intrathoracic space (Fig. a, b). On contrast imaging, the ileus tube in the transverse colon was observed to be incarcerated in the right intrathoracic space (Fig. c), a finding which was confirmed on computed tomography (CT) of the chest and abdomen (Fig. d). The patient was diagnosed with a DH and underwent laparoscopic hernia repair.
The surgical repair was performed with the patient in the left half-lateral decubitus position. Four laparoscopic ports were placed in the abdomen as follows: a 12-mm port at the umbilicus for the scope, two 12-mm ports at the right lateral lesion as working ports, and a 5-mm port at the middle of the upper abdomen used as an assistant. Under laparoscopic view, the transverse colon and small intestine anastomosis were found to be adherent to the right hemidiaphragm (Fig. a), and we proceeded with careful separation (Fig. b). After dense adhesions between the herniated organs and the pleural lining of right lung were sectioned, 5 × 3 cm diaphragmatic defect was observed, with the transverse colon found to be incarcerated in the defect and adherent to right lung (Fig. c, d). We proceeded with widening the diaphragmatic defect, gently peeling the transverse colon from the lung, and repositioning the transverse colon into the abdominal cavity. The damaged pleural lining of the lung (Fig. a) was repaired by a respiratory surgeon using a laparoscopic transdiaphragmatic approach (Fig. b). The diaphragmatic defect was confirmed to be different from the area repaired previously. It was then closed laparoscopically with non-absorbable 2–0 polyester sutures (Fig. c, d). The postoperative course was uneventful, and a contrast study of the ileus tube demonstrated good passage, with no leakage or stenosis. At 6 months after the surgery, all symptoms had disappeared.
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pmc-6243165-1
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A 32-yr-old female patient who had severe bloating, nausea, fatigue and abdominal pain for a week admitted to IBD Clinic of Behbood Research Center for Gastroenterology and Liver Diseases, Jan 2017. She traveled to abroad approximately 1 month before clinical symptoms and took prolonged antibiotic therapy for acute sinusitis. She had no previous history of any abdominal complaints. The blood tests revealed normal Complete Blood Count (CBC) consisted of White Blood Cell (W.B.C): 6600 /micL, Red Blood Cell (R.B.C): 4.7 mil/micL, Hemoglobin (Hb): 13.8 g/dl. Differential count of WBC was Neutrophils: 70%, Lymphocytes: 26%, Monocytes: 3% and eosinophils: 1%. Liver function tests such as SGPT, SGOT and Alkaline phosphatase were 44 U/L, 28 U/L, and 170 U/L, respectively. Celiac disease tests including IgA, EMA, tTg (IgA) were 180 mg/dL, Negative and 1.0 U/ml, respectively. IgA, EMA, and tTg (IgA) were analyzed by turbidimetry, Immunofluorescence (EUROIMMUN, Germany) and ELISA (ORIENTEC, Germany), respectively. Antigen of Helicobacter pylori was not detected in stool using rapid chromatographic immunoassay test. A fresh stool sample was taken and examined for intestinal parasites. Stool sample was loose, few greasy and yellow colored in the appearance.
Trophozoite of G. intestinalis was detected in direct examination (). RBC and PMNs were not seen in microscopical examination of stool sample. It was not seen cyst of G. intestinalis, and other eukaryotic parasites and also ova of helminths in formalin-ethyl acetate concentration.
Primary fecal calprotectin was determined using an ELISA-based kit (EUROIMMUN, Germany) and revealed a high elevation level of calprotectin (2047 mg/kg).
The patient was treated with metronidazole (250 mg/kg), omeprazole (20 mg) and bismuth (120 mg), three times a day for 10 days. After treatment period, stool sample was taken and secondary fecal calprotectin determination was performed that showed a significantly decreased level. The second stool examination showed that there were no trophozoite and cyst of G. intestinalis.
After treatment, the general condition was well and there were no clinical complaints.
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pmc-6243292-1
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Case 1: a 31-year-old female patient was referred for imaging for nasal block. The patient had history of 6–8 months of recurrent epistaxis but no history of headache or any systemic complaints. Clinical examination demonstrated a hard mass protruding through the right nostril (). Laboratory parameters, including levels of alkaline phosphatase, were normal. CT imaging of the nasal cavity was performed. Examination demonstrated enlargement of the posterior aspect of the basal lamella owing to a mass containing non-homogeneous areas of calcification. The lesion was occupying most of the mid-nasal cavity by displacing the inferior turbinate and extending into the nasal vestibule (). The patient underwent endoscopic surgery under general anaesthesia and complete excision of the mass was performed. The mass was adherent to the posterior aspect of the septum and the medial surface of the inferior turbinate. The resected specimen consisted of pieces of bony fragments, with the largest component measuring 3 × 2 × 1 cm. Microscopic evaluation demonstrated features suggestive of a benign fibro-osseous lesion, favouring FD ().
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pmc-6243292-2
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Case 2: a 32-year-old adult female presented with occasional nasal bleeding, difficulty in breathing and recurrent headaches. She had no visual complaints. Clinical examination was unremarkable. Nasal endoscopy revealed obliteration of the superior aspect of the left nasal cavity, with poor visualization of the details. Multidetector CT evaluation of the nasal cavity and skull was performed. CT examination revealed gross sclerosis of the frontal bone, orbital plates, zygomatic bone, ethmoid and sphenoid. The involved bone showed gross thickening of the inner and outer tables with obliteration of the normal architecture. There was uneven and disorganized structure of the skull bones involving the squamous part, orbital plate of the frontal bone, medial ethmoid, zygomatic bone and sphenoid. There was gross homogeneous enlargement of the lateral and basal lamella of the middle turbinate (). The whole length of the turbinate was involved, with ground-glass texture and preserved overall configuration. Owing to increase in the thickness of the turbinate and additional involvement of the medial wall of the ethmoidal sinus, the nasal cavity was completely occluded. There was significant narrowing of the superior orbital fissure. The optic canal and optic foramina were not obliterated. After due consideration of the cost and benefits of surgical intervention, the patient was advised to have regular check-ups. Surgical option was deferred for a later date, subject to progression of symptoms.
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pmc-6243294-1
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A 42-year-old male presented to the Department of Head and Neck Surgery of a tertiary oncological centre because of right mandibular swelling and trismus. The patient had already been admitted to a secondary care hospital 3 months earlier with complaints of right mandibular discomfort and slight tumefaction. A biopsy was then performed and the diagnosis of ameloblastic carcinoma was made histologically. The patient was otherwise healthy with no significant past medical history, including alcohol, smoking or tobacco abuse.
A complete head and neck examination revealed a painless, firm and fixed right mandibular mass with no cutaneous inflammatory signs. No ulcers or mucosal lesions were found in the oral cavity. Laboratory evaluation, chest radiograph and respiratory function tests were unremarkable. The patient underwent bronchofibroscopy, which revealed only mild laryngeal hyperaemia.
Both neck CT () and MRI () were performed, showing a large, solid tumour arising from the ramus and posterior body of the right mandible. The lesion extended to the surrounding soft tissues, with invasion of the masseter and medial pterygoid muscles and caused bulging of the buccal mucosa. The soft tissue component was hypointense on T1 weighted and hyperintense on T2 weighted MR images and showed avid enhancement after gadolinium administration on MR examination. CT scan disclosed striking sclerosis and irregularity of the mandibular ramus with some gas bubbles inside the medullary cavity and an expansive lytic component in the posterior body and angle with some bone-forming matrix inside. Prominent periosteal reaction was also identified, particularly in the outer cortical surface of the mandibular ramus with the typical pattern of a ruptured Codman triangle. No associated cystic lesion was found in the mandible. No enlarged lymph nodes were detected and the evaluation of the remaining cervical spaces was unremarkable. A thoracic CT scan was also performed, with no parenchymal lesions.
The patient underwent a right hemimandibulectomy and ipsilateral cervical lymph node dissection. Surgical resection also included the right submandibular gland and a segment of buccal mucosa that was swollen by the mandibular mass. Reconstruction was performed with free fibula graft. The surgical specimen included a large, white and solid tumour with 11 × 7.5 × 6.5 cm, corresponding to an invasive, moderately differentiated (G2) SCC (). Focal positive margins were found at the medial surface of the specimen. The resected buccal mucosa, submandibular gland and lymph nodes had no neoplastic tissue. Taking into account the imaging staging examinations and the post-surgical histological report, the final TNM stage was stated as pT3 N0 Mx. After surgery, the patient underwent adjuvant chemotherapy (cisplatin-based regimen) and radiotherapy.
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pmc-6243296-1
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A 33-year-old Asian female patient was referred to our hospital because of rapidly growing subcutaneous nodules in the right axilla. Her medical history was unremarkable, and there was no family history of BL or infection with Plasmodium falciparum malaria. The patient initially noticed a small non-pustular nodule in the right axilla. The axillary lymph nodes were not felt at that time. She was initially diagnosed with an atheroma by the referring physician and underwent excision of the skin lesion 4 weeks prior to admission. Pathological examination was not performed. Physical examination revealed a swollen, painful red lump on her right axilla. Complete blood count results were as follows: white blood cells: 6400 μl−1; lymphocyte count: 2163 μl−1; haemoglobin: 11.3 g dl−1; mean corpuscular volume: 88 fl; platelets: 208,000 mm−3. Biochemical profile was normal except for elevated levels of serum lactate dehydrogenase 1148 IU l−1 (normal: 105–333 IU l−1) and C-reactive protein 1.5 mg l−1 (normal: < 0.3 mg l−1). The patient was seronegative for anti-human immunodeficiency virus antibody and anti-Epstein–Barr virus immunoglobulin M and G. Axial contrast-enhanced CT scan showed a large, poorly marginated homogeneous soft tissue mass in the cutaneous and subcutaneous compartments of the right axilla, and the tumour was slightly enhanced after intravenous administration of contrast medium (). MRI of the right axilla was performed to further characterize the lesion using a 1.5 T unit (Signa HDxt 1.5 T, GE Healthcare, Waukesha, WI). Coronal T1 weighted MRI revealed a homogeneous mass of slightly increased signal intensity compared with normal muscle (). On T2 weighted MRI, both the subcutaneous tumour and the axillary lymph nodes had intermediate signal intensity (). Fat-suppressed contrast-enhanced T1 weighted MRI showed homogeneous contrast enhancement (). Skin thickening and marginal septal enhancement were also present. The subclavian vein and artery were not invaded or encased by the tumour. From these findings, the differential diagnoses of this mass were lymphoma, melanoma, breast cancer, fibrosarcoma and malignant peripheral nerve sheet tumour. Excisional biopsy of the axillary mass revealed an enlarged lymph node with “starry sky” appearance owing to abundant macrophages. Immunohistochemical and flow cytometric evaluation showed strong expression of CD20, CD10, CD19, CD22 and surface κ immunoglobulin light chain, but weak or no expression of CD3, Ki-1, bcl-2, cyclin D1 and CD34. Based on these findings, a diagnosis of BL was made. The patient received cyclophosphamide (200 mg m−2 day−1, days 1–5), doxorubicin (50 mg m−2 day−1; day 5), vincristine (1.3 mg m−2 day−1; day 2), dexamethasone (10 mg m−2 day−1 orally; days 1–5, tapered in 1 week), followed by intrathecal chemotherapy with 15 mg of methotrexate and 4 mg of dexamethasone. The patient achieved complete remission and received eight courses of maintenance therapy consisting of cyclophosphamide, doxorubicin, vincristine and dexamethasone. She remains in complete remission at 3 years after the treatment.
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pmc-6243302-1
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A 66-year-old white female with a 50 pack-year smoking history presented to our tertiary hospital with acute massive haemoptysis. She had recently moved interstate, with no patient data available in our hospital system. The patient also reported anorexia and weight loss of approximately 3 kg in the past 6 months. On examination, she was afebrile, pulse rate was 90 beats min–1, respiratory rate was mildly increased at 22 breaths min–1 and blood pressure was slightly elevated at 150/90 mmHg. On auscultation of the chest, fine rales over the right lung base were noted. Laboratory results showed normal white blood cell count at 7300 cells l–1 (3.5–11 × 109 cells l–1), C-reactive protein 14 mg l–1 (< 5 mg l–1) and haemoglobin 100 g l–1 (110–165 g l–1). Her initial chest X-ray showed an opacity in the right lower lobe (RLL) contiguous with the right hemidiaphragm (). A CT pulmonary angiogram demonstrated a 2.8 cm solid enhancing nodule in the posterobasal segment of the RLL with a density measuring 35 HU, with a 7 mm central focus of dense calcification (); also noted were hyperdense endobronchial material in the RLL, which was thought to represent fresh blood. A suspicion of lung cancer was raised, especially in view of heavy smoking history and reported weight loss. Positron emission tomography revealed increased 18F-fludeoxyglucose (FDG) uptake [maximum standardized uptake value (SUVmax) of 5] within the RLL nodule; the report described the finding as concerning for malignancy, with the differential diagnosis of an inflammatory pseudotumour (). A subsequent bronchoscopy was complicated owing to active bleeding from the RLL bronchus. The transbronchial biopsy showed no malignant cells, acid-fast bacilli or fungi.
Upon targeted questioning, the patient disclosed having had a complicated cholecystectomy 3 years ago, performed at another hospital, with attempted laparoscopic cholecystectomy converted into an open laparotomy owing to gallbladder rupture with intraperitoneal spillage of gallstones; this was complicated by the formation of post-operative subphrenic abscess, which was surgically drained. The relevant externally performed images have been retrieved, including a CT scan of the abdomen (). The patient further admitted to occasional episodes of minor haemoptysis of about two spoonfuls over the past 2 years, associated with right-sided mild chest pain, which she did not seek medical attention for.
As the RLL mass was the presumed cause for the repeated episodes of haemoptysis, a thoracotomy was recommended and the patient underwent a RLL wedge resection. A firm rhomboid-shaped calculus measuring 11 × 7 × 8 mm () that dislodged from the specimen was confirmed to be a gallstone. Pathological examination additionally found abundant bile pigment (25%) surrounded by microorganisms, extensive interstitial fibrosis and hyalinization. Further biochemical analysis of the calculus revealed the presence of 85% cholesterol. Post-operative recovery was uneventful, and the patient was discharged from the hospital in a satisfactory condition 1 week after the surgery.
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pmc-6243304-1
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A 16-year-old male patient came to our hospital with a history of left flank pain for 3 months, two to three episodes of gross haematuria and abdominal distension for the preceding 2 weeks. Pain was described as a dull ache, non-radiating in nature, and not associated with fever or burning during micturition. On examination, the patient was of average built, having no icterus or pallor, and had distension of the abdomen with shifting dullness. Serum glutamic oxaloacetic transaminase (49 U l−1), serum glutamic pyruvic transaminase (37 U l−1) and bilirubin (0.6 mg%) levels were normal, and prothrombin time was prolonged (20 s). Blood haemogram and renal function tests were within normal limits.
Transabdominal ultrasound imaging revealed a hypoechoic mass in the left kidney in the interpolar region, with extension of the tumour to the left renal vein and inferior vena cava (IVC). Contrast-enhanced CT scan of the abdomen showed a large lobulated heterogeneously enhancing mass of size 10.5 × 7 × 5 cm arising from the left kidney with infiltration of adjacent pararenal fat and the retroperitoneum (). Enhancing tumour thrombus was seen extending into the IVC through the left renal vein in continuity with the primary renal mass. Superiorly, the thrombus extended into the intrahepatic portion of the IVC and the right hepatic vein, causing the Budd–Chiari syndrome (). There was homogeneous enhancement of the liver parenchyma with normal opacification of the left and middle hepatic veins, and the portal vein. There was mild ascites. An imaging diagnosis of Stage III (T3bN0M0) renal cell neoplasm was made. Biopsy of the mass demonstrated monomorphic small, round cells arranged in a sheet-like pattern with round nuclei, scanty eosinophilic cytoplasm and indistinct cell outline (). Immunohistochemistry revealed positivity for cluster of differentiation 99 (MK2), vimentin and synaptophysin (). Based on these findings, a diagnosis of renal ES/PNET was established.
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pmc-6243305-1
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A 73-year-old female from a rural region of north India presented with vague abdominal pain, burning micturition and urge incontinence. She gave a history of intermittent passage of small, white, balloon-like, grape-sized structures in the urine for the past 6 months. Her previous medical and family history were unremarkable. Physical examination revealed a visible lump in the epigastric region on the right side and a palpable lump in the left flank. All biochemical and haematological parameters were normal. Chest X-ray was normal. On ultrasonography (USG), two adherent multicystic intraperitoneal lesions were seen occupying the right hypochondrium and the epigastric region (), and others were seen in the left lumbar region, right adnexum, right iliac fossa and retrovesical region. On MRI, the lesion in the liver appeared hypointense and those in the hypochondrium appeared as multicystic hyperintense lesions (). The large cystic lesion in the retrovesical location contained free-floating daughter cysts and communicated with the posterior wall of the urinary bladder (). On MRI, these lesions were characterized as multicystic lesions in respective locations and the retrovesical cyst showed fistulous communication with the urinary bladder (). On gross examination of urine, a single balloon-like membranous cyst was seen and histopathological examination showed an outer laminated layer with an inner germinal layer, which was consistent with a hydatid cyst. The serology for hydatid disease was not positive in our case. Based on USG and MRI findings, a diagnosis of disseminated intraperitoneal hydatidosis with hepatic and retrovesical cysts was made. The patient was referred to the urology department for further management.
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pmc-6243312-1
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The patient is a 45-year-old female who was referred to the radiology department from the regional cancer center for imaging evaluation of a sonographically detected ovarian carcinoma. She was asymptomatic for the pancreatic lesion. She underwent CT imaging as a part of routine follow-up, which identified a pancreatic lipoma. Ultrasound and MRI were performed subsequently. MRI corroborated the CT scan finding.
On ultrasound (), the lesion was iso to hypoechoic when compared with liver echogenicity and located on the head of the pancreas. It appeared as a soft lesion on the elastographic grayscale image. The rest of the pancreas was normal in size and echogenicity, without significant dilation of the main pancreatic duct.
Plain and contrast sections of the CT scan of the abdomen ( and ) showed bilateral enhancing adnexal lesions; a well-defined, lobulated, homogeneous fat density lesion of approximately 3.5 cm (transverse) × 1.9 cm (anteroposterior) × 3.5 cm (craniocaudal length) on the head of the pancreas without infiltration of peripancreatic fatty tissue; and widening of the pancreatic duct and common bile duct.
MRI of the abdomen was performed to confirm its benignity, as it was a leave-alone lesion. T (), T () and T1 fat-suppressed images () were taken. It was hyperintense on T1 and T2 images. T1 hyperintensity was suppressed on fat-suppressed sequences, confirming the fatty nature of the lesion.
The patient was being followed up for ovarian cancer. Prior CT scans had already revealed the pancreatic lesion and when compared with the recent scan, the size of the lesion appeared stable, suggesting benignity. Therefore, histological confirmation was not obtained.
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pmc-6243314-1
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A 54-year-old male had been experiencing chronic pain in his chest, back and both legs for 3 years. He was found to have hypophosphataemia and a high serum alkaline phosphatase level, and was referred to our hospital for further examination and treatment. Laboratory tests showed low serum phosphorus (2.0 mg dl−1), elevated serum alkaline phosphatase (933 IU l−1) and FGF23 (96.3 pg ml−1), and high urinary phosphorus (1.8 g day−1) levels. Based on these findings, tumour-induced osteomalacia such as PMT, which is associated with FGF23 secretion, was suspected. Systemic venous sampling for FGF23 analysis was performed. However, tumour localization was not successful.
CT scan showed a low-density tumour with a well-defined sclerotic margin in the anterior aspect of the L5 vertebra (). On MRI, pre-contrast T1 and T2 weighted images revealed decreased signal intensity compared with the vertebral body. The tumour showed heterogeneous enhancement (). For 68Ga-DOTATOC PET/CT scan, 108.3 MBq of 68Ga-DOTATOC was injected intravenously and whole-body PET/CT scan was performed. The 68Ga-DOTATOC PET/CT scan demonstrated intense focal uptake within the tumour (maximum standardized uptake value = 10.5) (). The scan did not show any abnormality in other regions. Surgical excision of the tumour was performed. Histological examination of the sections revealed proliferation of oval-to-short spindle-shaped cells arranged in sheets or a haphazard pattern, accompanied by fibrocollagenous stroma, abundant various-sized vessels, microcysts and thickened anastomosed bone trabeculae. Immunohistochemically, the tumour cells were focally positive for FGF23 (not shown). The final diagnosis of PMT was confirmed in conjunction with the serological elevation of FGF23. The postoperative course was uneventful. The patient experienced a significant decrease in systemic bone pain and the laboratory data normalized immediately.
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pmc-6243315-1
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A 73-year-old male with a past medical history of chronic right pleural effusion, restrictive ventilatory impairment and hypertension presented for evaluation of severe right chest pain of few days’ duration and severe dyspnoea.
Upon arrival, he was haemodynamically stable with a rhythmic heart rate of 98 beats per min, blood pressure of 155/70 mmHg and respiratory rate of 38 per min. His oxygen saturation was 94% on room air.
Physical examination showed that the patient was awake and orientated but cyanotic and dyspnoeic. Upon auscultation, it was noted that breath sounds were completely absent in the right hemithorax and there was diffuse inspiratory/expiratory whooping in the contralateral hemithorax. All these physical findings were predictive of pneumothorax and the patient was admitted to the department of radiology for evaluation.
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pmc-6243317-1
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A 59-year-old female presented to our emergency department with upper quadrant pain and vomiting. On physical examination, severe epigastric tenderness was present but the abdomen was not distended. The patient complained of weight loss of 5 kg and abdominal pain, which had progressively gotten worse over the previous 2 months, becoming continuous, increasing after meals and being relieved only after vomiting. There was no history of alcohol abuse. Blood sample test results revealed increase in amylase (156 U l−1; upper reference value 100) and cancer antigen 19-9 (52 U ml−1; upper reference value 37) levels, with normal complete blood counts.
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pmc-6243319-1
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A 40-year-old male, suffering from diabetes mellitus for the past 2 years and on irregular treatment, was brought in with a history of progressive jaundice of 10 days’ duration, and fever and right upper abdominal pain for the past 3 days. There was also a history of passage of clay-coloured stools. The patient mentioned occasional post-prandial self-limiting right upper quadrant pain in the past, for which he did not get himself investigated. On examination, he was found to be febrile with a temperature of 102 °F and appeared dehydrated. His pulse was 100 min−1, regular but low in volume, with a blood pressure of 110/70 mmHg and respiratory rate of 18 min−1. He had marked icterus. Murphy’s sign was negative. An ill-defined, sausage-shaped, tender parasagittal supraumbilical mass was palpable on the right side. Erythema was noted on the skin around the umbilicus; however, there was no umbilical discharge. Laboratory investigations revealed polymorphonuclear leukocytosis (total leukocyte count 19,000 mm–3 ), with elevated liver enzymes (alkaline phosphatase 400 IU l−l, alanine transaminase 90 IU l−l, aspartate transaminase 100 IU l−l) and conjugated hyperbilirubinaemia (16 mg dl−1). His blood glucose was 250 mg dl−1. The rest of the biochemical tests were normal.
Transabdominal ultrasonography of the abdomen revealed cholecystolithiasis. However, there were no signs of cholecystitis. Bilobar dilatation of the intrahepatic biliary radicles was noted and the common bile duct measured 20.0 mm in diameter. A calculus measuring 8.2 mm was seen in the middle segment of the common bile duct. A tubular cystic structure with echogenic debris was visualized, extending from the umbilicus off midline on the right side to the inferior surface of the medial segment of the left lobe of the liver. A subsequent MR cholangiopancreatography (Symphony Tim 1.5 T MRI scanner, Erlangen, Germany) also showed cholecystolithiasis and choledocholithiasis with an 8.0 mm-sized calculus in the distal part of the supraduodenal common bile duct. The entire biliary tree proximal to the calculus was dilated. A sausage-shaped cystic lesion with thick shaggy walls was seen on the right side, extending from the umbilicus cranially up to the umbilical fissure. This lesion measured 16.0 × 3.5 × 3.5 cm (craniocaudal × anteroposterior × transverse). The normal flow void in the left branch of the portal vein was replaced by hyperintense signal intensity (–). These findings were consistent with cholecystolithiasis, obstructive choledocholithiasis with cholangitis, left portal vein thrombosis and an abscess of the ligamentum teres hepatis.
Under ultrasound guidance, 80 ml of pus (which later grew Escherichia coli on culture) was aspirated from the abscess on the day of admission and the patient was started on broad-spectrum intravenous antibiotics and insulin. He was also started on injectable low-molecular-weight heparin. The next day, he underwent endoscopic retrograde cholangiopancreatography with sphincterotomy and stone extraction. The patient gradually recovered with subsidence of fever and improvement of the clinical parameters. On the tenth day post admission, he underwent laparoscopic cholecystectomy and excision of the residual abscess cavity. The patient made steady recovery after the surgery and was discharged on the seventh post-operative day. Ultrasonography performed on the day of discharge was normal except for evidence of cholecystectomy. Colour Doppler flow imaging revealed normal colour flow in the left portal vein. On follow-up, the patient remained asymptomatic with well-controlled blood sugar and normal liver function.
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pmc-6243320-1
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A 72-year-old male was admitted to a tertiary academic hospital because of sudden severe central abdominal pain. Except for hyperlipidaemia, no abnormal laboratory findings such as lactate, white blood cells, amylase, lipase and liver enzymes were present. His vital signs were normal. His medical history included atrial fibrillation and Type 2 diabetes mellitus. He had not taken warfarin for 2 weeks. Contrast-enhanced CT angiography (CTA) revealed segmental occlusion of the mid portion of the main trunk of the SMA proximal to the ileocolic artery (). Proximally and distally from the occlusion SMA was of normal size, suggesting an embolism rather than a thrombosis. No signs of irreversible bowel wall ischaemia such as bowel wall thickening or pneumatosis were evident. It was considered that thrombolysis could not be effective because of the largesize of the embolus. Because the embolus was located in the main trunk of the SMA, it was expected that aspiration embolectomy would be effective.
After careful consideration, we obtained consent from the patient under an institutional review board-approved protocol for using a 5MAX ACE reperfusion catheter for endovascular revascularization. A decision for endovascular treatment via the transfemoral approach under local anaesthesia was reached in concordance with the surgeons.
Access was established via the right common femoral artery and a 6 French introducer sheath system (Terumo Corporation, NJ). Selective catheterization of the SMA was performed using an angled glide catheter (4 French; Cordis Corporation, Miami Lakes, FL) and a 0.035-inch long guidewire (Radifocus Guidewire M; Terumo Corporation, Tokyo, Japan). Initial SMA angiography confirmed acute occlusion in the mid portion of the SMA trunk (). After infusing 4000 IU of heparin, the 5MAX ACE reperfusion catheter exchange was performed and it was advanced to the face of the clot over the 0.035-inch guidewire. Once the 5MAX ACE was immediately adjacent to the clot, the guidewire was removed. Mechanical suction was applied to the 5MAX ACE using a Penumbra aspiration pump and aspiration tubing (Penumbra, Inc., Alameda, CA) (). Once a good seal was obtained, the 5MAX ACE was slowly withdrawn, while maintaining aspiration. Afterwards, the 5MAX ACE was removed from the body, and the guide sheath was opened to enable back bleeding of possible clots. The catheter was then flushed forward once it was confirmed that there were no more clots. Final contrast injection after five passes demonstrated near complete resolution of the thrombus from the main trunk of the SMA (). No adjunctive endovascular procedure was applied. The patient’s symptoms almost immediately improved after the procedure, and he no longer complained of postprandial abdominal pain. He remained admitted after the procedure for 3 days and was instructed to continue taking dabigatran etexilate after discharge. CTA on the day after the procedure revealed that the thrombus of the SMA had resolved. A 3-month follow-up excluded any clinical symptoms of abdominal ischaemia.
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pmc-6243321-1
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A 24-year-old female presented to the gastroenterology department with non-specific abdominal pain for 10 weeks with four episodes of haematemesis during this period. The abdominal pain was mild and non-localized, with no specific aggravating factors. She also reported lack of appetite. She denied any history of fever, cough or weight loss.
Physical examination was unremarkable. No signs of portal hypertension were present. Her haemoglobin, thrombocyte and white blood counts were 7.9 g dl–1, 112,000 mm−3 and 8010 mm–3, respectively, with high lymphocyte count. Erythrocyte sedimentation rate was elevated (112 mm hr–1). Human immunodeficiency virus test, serological investigations for hepatitis and cultures of blood and urine were negative. Renal function tests and hepatic transaminase levels were in the normal range. Chest radiograph was normal.
The patient was initially treated symptomatically with antispasmodics and was sent to the radiology department for ultrasonography and abdominal multidetector CT (MDCT) scan to further work-up the aetiology of the abdominal pain and haematemesis.
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pmc-6243323-1
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A 53-year-old female presented to the ER with chief complaints of dysphagia, odynophagia, breathlessness and chest pain. She gave a history of accidentally swallowing some FB while taking her regular medication, which was now giving her a sensation of something stuck in her neck. On examination, her neck, face and eyelids were swollen, and she had subcutaneous crepitus on palpation. She had a dental repair performed 16 years ago, with metallic dentures fitted in both upper and lower jaws.
A skull radiograph was taken, which showed a missing partial denture from the right lower jaw ().
Anteroposterior and lateral chest radiographs () showed two metallic density objects in the retrocardiac area adjacent to the descending aorta with mild right-sided pleural effusion, pneumomediastinum and subcutaneous emphysema.
Clinically, the suspicion of a perforated oesophagus was raised and CT imaging of the neck and thorax was ordered to confirm the diagnosis. On the CT scan, two metallic density objects (measuring approximately 17 mm each) were seen in the middle one-third of the oesophagus (), with a suspicious contained leak of orally ingested positive contrast media along the right posterolateral aspect of the oesophagus (). In addition, there was pneumomediastinum with left pneumothorax and subcutaneous emphysema of the neck and chest, which confirmed the diagnosis of oesophageal perforation secondary to ingested dentures ().
The patient was haemodynamically stable and was rushed for emergency thoracoscopic removal of the FB. Under thoracoscopic guidance, a rent of 2 cm and an ingested partial denture were confirmed in the right posterolateral aspect of the mid-oesophagus. The denture () was removed and the oesophageal tear was repaired subsequently.
The patient was shifted to post-operative intensive care facility and started on broad-spectrum antibiotics. The post-operative period was uneventful. A follow-up oral gastrograffin study was performed on post-operative day 7, which revealed no leak. Per oral diet was then started gradually ().
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pmc-6243330-1
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A 31-year-old male presented to our emergency department with right urinary tract stones. He had no medical history of note and was on no medications. Physical examination of the abdomen was unremarkable. Vital signs and laboratory data were within normal limits.
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pmc-6243333-1
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A 42-year-old male was brought to our emergency room with an episode of loss of consciousness for half hour. The patient had been complaining of headache, blurring of vision in the left eye and vomiting for the past 1 month. He was drowsy, confused and irritable. His general physical examination was normal. He had relative afferent pupillary defect in the left eye. Optic fundus examination showed bilateral papilloedema. Visual assessment performed subsequently showed no perception of light in the left eye and a visual acuity of 6/24 in the right eye.
CT scan showed a large heterogeneous ill-defined lesion in the suprasellar region, which extended into the left basifrontal region and through the left optic canal into the left orbit. The optic nerve sheath complex appeared thickened and tortuous, and showed peripheral tram-track-like calcification with a widened optic canal. Minimal hydrocephalus was seen ().
MRI of the brain showed a large heterogeneous ill-defined lesion seen in the suprasellar region, which extended superiorly into the left basifrontal region and anteriorly through the left optic canal into the left orbit. The left optic nerve appeared thickened and showed tram-track calcification. The optic tract and the chiasma were not seen separately from the lesion. The lesion was seen exerting significant mass effect on the anterior aspect of the left lateral ventricle and the floor of the third ventricle, causing obstructive hydrocephalus and cerebral oedema. Posteriorly, the lesion was seen extending into the interpeduncular cistern ( and ). The patient suffered a seizure during the MRI; hence, contrast-enhanced MRI could not be performed. Diagnoses of optic pathway glioma and meningioma were considered.
The patient was taken up for emergency surgery. Left frontal craniotomy with decompression of the tumour was performed and sample was sent for histopathology.
The tumour was very vascular and had variable consistency with areas of gritty calcification. Areas of necrosis filled with dark brown-coloured fluid were seen. A ventriculoperitoneal shunt tube was placed for relieving the hydrocephalus. He was then treated with intensity-modulated radiotherapy and chemotherapy with temozolomide. The lesion turned out to be an anaplastic oligoastrocytoma on histopathological examination ().
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pmc-6243336-1
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In April 2015, a 58-year-old male with a history of recurrent melanoma, currently Stage IV, was admitted to our RT department for back pain owing to bone lesions at the T10–T12 vertebral levels. No peripheral neurological symptoms were present but vertebral lesions were at a high risk of fracture with consequent spine compression. In February 2009, he was diagnosed with two nodular melanomas in his back, which were treated with local excision. Thereafter, he remained free of disease until March 2015. At that time, he underwent CT/positron emission tomography scans for persistent pain in the lower back region with impaired deambulation, which was treated using anti-inflammatory drugs with no clinical benefit. The CT/positron emission tomography scans showed multiple metastatic lesions (brain, bone, lymph nodes and skin). Biopsy from a skin metastasis site revealed a BRAF V600E-mutated melanoma. Therefore, systemic therapy with dabrafenib was started at a standard dose (150 mg twice daily) while it was planned to start the MEKi (trametinib) after 2 weeks within the expanded access program. In our patient, trametinib was administrated about 5 weeks after the end of radiation course.
For his bone lesions (T10–T12 and T7 vertebrae), the patient was soon scheduled for RT at a dose of 30 Gy administered in 10 fractions (3 Gy per fraction for 5 days a week). Because of a rapidly evolving disease, which needed a rapid and hopefully consistent response, dabrafenib was not interrupted during RT. Two different 3D conformal RT techniques were used: an isocentre technique with two oblique wedge pair fields for the T7 lesion and a direct skin–source distance posteroanterior field for the T10–T12 vertebrae using an 18 MV linear accelerator (). After six fractions of RT (18 Gy), an increasing, unexpected skin toxicity appeared in the field of irradiation at the T10–12 level, both on the back and the abdominal region (). This acute side effect was classified as Grade 2 radiodermatitis [according to the Common Terminology Criteria for Adverse Events (CTCAE) version 4.0]. No acute skin toxicity or other systemic toxicity were documented in the field of the T7 vertebra. To further understand why the skin toxicity occurred in only one of the irradiated fields, the dose distribution of the two different RT treatment plans was reviewed. The absorbed doses to the target volumes (90% of the volume absorbed 95% of the prescribed dose for both volumes) and the maximum dose were found to be similar for the two plans. On the contrary, the volume of subcutaneous tissues that received a high dose was found to be significantly larger for the T10–T12 field than for the T7 field. In particular, the mean doses, V10, V15 and V20 (volume that absorbed 10, 15 and 20 Gy, respectively) were 78 and 33 cm3, 65 and 12 cm3 and 13 and 7 cm3, respectively, for T10–T12 and T7 (). Moreover, 50 cm3 of subcutaneous tissues absorbed 16.5 and 8.7 Gy for the T10–T12 and T7 field, respectively. The subcutaneous tissues of lateral and anterior chest wall absorbed a mean and maximum dose of 14 and 18 Gy, and 7.5 and 8 Gy for T10–T12 and T7, respectively, confirming that the exit dose washigher for the T10–T12 than the T7 field. Owing to this toxicity, after a multidisciplinary discussion, the radiation course was stopped at a total dose of 18 Gy for both the irradiated volumes. At the 3-month follow-up, the patient had a significant pain reduction without the appearance of neurological symptoms and a new CT scan revealed a stable osseous disease.
From the clinical point of view, these findings open the discussion to whether the acute skin toxicity caused by the treatment with BRAFi in association with RT should be prevented by reducing high dose areas to the skin and the subcutaneous tissues.
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pmc-6243340-1
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A 41-year-old male was found to have a large abdominal mass that was confirmed through biopsy to be a desmoplastic small round cell tumour (DSRCT). A high power 400× histological image of the routine preparation is shown (). Microscopic examination showed the presence of epithelioid-like cells with a high nuclear–cytoplasmic ratio with moderate variability in size and shape. Cytologically, the tumour cells were intermediate in size with polarized, slightly scalloped nuclei and a small amount of eosinophilic cytoplasm. Infrequent mitotic figures were seen and geographic areas of necrosis were identified. Immunohistochemical staining revealed positivity to vimentin and staining with desmin showed a peculiar perinuclear dot pattern characteristic of DSRCT. CD56 was strongly positive, WT1 focally positive and other markers, including CD117, CD99, CD45, CD34, CD20, Cam 5.2, pancytokeratin, MYF4, S100, Actin, CD138, calretinin and synaptophysin, were negative. MIB-1 staining showed a high proliferative index with 60% positivity.
CT imaging showed an abdominal mass measuring 21 × 11 × 14 cm and the patient was treated with one cycle of chemotherapy with cyclophosphamide, adriamycin, vincristine, ifosfamide and etoposide; however, his mass progressed to 25 × 16 × 22 cm. 18F-fludeoxyglucose positron emission tomography (18F-FDG PET)/CT scan showed a mostly necrotic 25-cm abdominal mass with a maximum standardized uptake value (SUVmax) of 18.5 with limited peritoneal disease (left pelvic nodule measuring 2.0 × 1.4 cm with SUVmax of 13.9) ( and ), which altered the treatment strategy from curative to palliative, and palliative radiation therapy (RT) was initiated. The patient received 5000 cGy to 90% of the planning target volume in 25 fractions over 6 weeks. Post RT 18F-FDG PET/CT scan performed 4 weeks after the end of therapy showed an excellent treatment response (abdominal mass SUVmax of 18.5 decreased to 7.7, pelvic mass SUVmax of 13.9 decreased to 2.4) ( and ). Based on the excellent response to therapy, as determined by the PET/CT scan, the patient opted for an aggressive approach with an attempt at curative resection of the tumour with peritonectomy and heated intraperitoneal chemotherapy (HIPEC), which was performed with cisplatin, 50 mg m−2 of body surface area (92.5 mg) in 4.5 l of 1.5% dianeal solution. The tumour was found to be arising from the transverse colon mesentery and was completely resected along with the left pelvic nodule. Unfortunately, a post-operative PET/CT scan performed 6 months after the surgery showed new peritoneal lesions (perisplenic soft tissue SUVmax of 18.2) and numerous bone metastases in the left humerus, cervical and thoracic spine, and ribcage ( and ). He had more RT from C7 to T8, and a follow-up PET/CT scan 3 months later showed extensive soft tissue progression (). The patient refused further chemotherapy and passed away a few months later (20 months post-histological diagnosis, 12 months post-surgery/HIPEC).
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pmc-6243341-1
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A 71-year-old female presented to an ear, nose and throat specialist in November 2014 complaining of obstructive dysphagia for 4 months, without pain. She did not report haemoptysis or other clinical signs or symptoms.
During the clinical examination, the physician found a bulky mass localized to the left tonsillar bed, without the presence of mucosal ulceration. There was no clinical evidence of any pathological cervical lymph nodes. A maxillofacial CT scan and MRI were then scheduled.
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pmc-6243345-1
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A 22-year-old female was admitted to our hospital with weakness in her left hand. When she was 20 years old, she began experiencing difficulty with moving her right hand while changing her clothes. MRI of the brain showed hyperintensity of the left corticospinal tract on T2 weighted images. The symptom improved slightly without treatment. When she was 22 years old, she began to feel numbness in both hands and weakness in her left hand. Subsequently, the numbness spread to both upper extremities.
Neurological examinations showed atrophy of both hands and feet, decreased muscle tonus and loss of tendon reflexes of the bilateral upper and lower extremities. Loss of sensation in the right side of the body and face was also noted. Conduction velocity of motor and sensory nerves decreased bilaterally in the upper extremities (motor nerve conduction velocity 25.7 m s–1, sensory nerve conduction velocity 19.3 m s–1 and compound muscle action potentials 10.8 mV in right median nerve). Galactosylceramidase (GALC) activity in white blood cells decreased to 0.17 nmol mg–1 h–1 (reference value: 1.75–8.23 nmol mg–1 h–1), and the patient was diagnosed with adult-onset Krabbe disease. A genetic test for GALC mutation was not performed.
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pmc-6243345-2
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A 50-year-old female who had previously been diagnosed with adult-onset Krabbe disease was admitted. She had experienced bilateral upper and lower extremity weakness since her 30s. Her symptoms had slowly progressed and neurological examinations when she was 40 years old revealed atrophy of muscles in bilateral lower and upper extremities, loss of superficial sensation in the right hand and both feet and a spastic waddling gait. The conduction velocity of the motor and sensory nerves had decreased bilaterally in the upper and lower extremities. T2 weighted MR images of the brain showed hyperintensities along both corticospinal tracts. The decrease in the white blood cell GALC activity to 0.053 nmol mg–1 h–1 (reference value: 1.93–5.58 nmol mg–1 h–1) was indicative of adult-onset Krabbe disease. A genetic test for GALC mutation was not conducted. When she reached 50 years of age, the weakness had progressed further, and she was admitted to our hospital for rehabilitation.
Neurological examinations showed bilateral atrophy in the upper and lower extremities. Her muscle tonus was flaccid in the upper extremities and spastic in the lower extremities. Bilateral sensory deficits were detected in the upper and lower extremities. A nerve conduction study revealed further decrease in the nerve conduction velocity compared with that recorded previously (compound muscle and sensory nerve action potentials were not detectable in bilateral median nerves).
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pmc-6243347-1
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A 66-year-old female, who was previously well, presented with a 2-week history of night sweats, new onset headache, dizziness, intermittent double vision and unsteady gait. On physical examination, she walked with a left-sided hemiplegic droop, leaning towards the left side. She was initially referred for an MRI which, on T1 weighted post-gadolinium images, showed several lesions in the right temporal lobe, the largest measuring up to 2.4 × 1.9 cm with intense irregular ring-like enhancement ( and ). The lesions were associated with T2 hyperintensity and the differential diagnoses of the MRI findings included high-grade astrocytoma, lymphoma, metastases and infection, and the patient was referred for an 18F-fludeoxyglucose (FDG) positron emission tomography (PET)/CT scan (Discovery ST, GE Healthcare, Waukesha, WI) to assess for possible malignancy. She was injected with 12 mCi of 18F-FDG and imaged approximately 75 min after the injection. The PET/CT scan failed to show extracerebral 18F-FDG-avid lesions, and the intensely enhancing cerebral lesions described on MRI were hypometabolic [maximum standardized uptake value (SUVmax) 5.4] when compared with normal grey matter (SUVmax 10.1 for the basal ganglia and 7.6 for the sensorimotor cortex) ( and ), which increased the likelihood of an infectious aetiology. Following the failure of a diagnostic/therapeutic trial of antimicrobials for toxoplasmosis, a brain biopsy and culture confirmed the presence of Histoplasma capsulatum. The patient was also diagnosed with human immunodeficiency virus (HIV) infection and was put on highly active antiretroviral therapy. Her central nervous system (CNS) histoplasmosis was treated with i.v. liposomal amphotericin B (at a dose of 3 mg kg−1 day−1) and her neurological symptoms gradually improved. A 2-year follow-up MRI showed complete resolution of the CNS histoplasmosis lesions ().
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pmc-6243348-1
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A 28-year-old G3 P1 SAB1 female with no prior health concerns was found to have an abnormal integrated maternal serum screen indicating a 1 : 7 risk for trisomy 18. Foetal ultrasound at 19 weeks gestation revealed short femurs (< 2.5%) and an otherwise normal examination. A multidisciplinary approach was taken, including a genetics consult. The family history was notable for one nephew with “weak bones”. The patient and her husband reported that they were of Yemeni ancestry and distantly related. A recommended amniocentesis was performed, which showed increased α-fetoprotein at 2.26 MoM and absence of an acetylcholinesterase band. Chromosome analysis revealed a normal male karyotype (46,XY). Follow-up ultrasound at 22 weeks gestation again demonstrated short femurs, measuring 3.5 cm (< 2.5%) (). Biparietal diameter, head and abdominal circumference measured between the 39th and 55th percentiles. The patient chose to forgo further follow-up with genetics, and the remainder of the pregnancy was otherwise uneventful.
The baby was born by C-section at 37 4/7 weeks gestation owing to foetal decelerations. Upon delivery, the infant was found to have hoarse cry, weak reflexes and low tone. Multiple dysmorphic features were discovered, including short humeri and femurs; bowed lower legs; narrow chest; large ear lobes; retrognathia; yellowish hypertrophic gums and a low, flat palate; hypertrichosis of the bilateral temporal region; and light hair colour that was atypical for his ethnic background. He exhibited diffuse patchy ecchymoses on the trunk and persistent thrombocytopenia as well as hyperbilirubinaemia. Echocardiogram showed a small atrial septal defect and a large patent ductus arteriosis. The infant also experienced respiratory distress, requiring continuous positive airway pressure ventilation.
Radiological investigation at that time revealed the following:
diffuse demineralization of bony structures ()
profound diaphyseal cloaking of the long bones ( and )
relatively short humeri and femora ()
poorly formed and irregular appearing proximal humeral and femoral metaphyses ( and )
thickened and poorly formed clavicles ()
thickened and shortened ribs with an abnormally increased cardiothoracic ratio ()
poorly formed iliac bones with flattening of the acetabular roofs ()
unusual bowing of the distal ulna and radius with metaphyseal cupping ()
thickening of the proximal phalanges and minimal narrowing at the proximal aspect of the metacarpal bones.
Genetics service was consulted. Tests revealed elevated levels of multiple plasma and leukocyte lysosomal hydrolases, consistent with a diagnosis of I-cell disease. GNPTAB gene analysis revealed homozygous c.376_379delTTAG deletion mutations. This deleterious mutation has not been reported previously in individuals with I-cell disease.
The patient was eventually discharged to the care of hospice and passed away at 5 weeks of age. Additional information regarding the case was limited as an autopsy was declined by the family.
However, the parents did follow-up with genetics several months later for future family planning. When the wife became pregnant again later that year, chorionic villous sampling was obtained at 10 weeks gestational age and sent to test for I-cell disease. Uridine diphosphate-N-acetylglucoseamine-1-phosphotransferase enzyme activity was low, consistent with I-cell disease. Soon after obtaining these results, the family chose to terminate the pregnancy at 15 weeks. Chorionic villous testing was again obtained during a fifth pregnancy, and testing revealed that this child was neither affected nor a carrier of the abnormal GNPTAB gene. This child was born at full term without any health concerns.
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pmc-6243350-1
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A 45-year-old female was diagnosed with HIV infection in December 2013. She was started on highly active antiretroviral therapy comprising tenofovir 300 mg once daily (OD), lamivudine 300 mg OD and nevirapine 200 mg twice daily 5 months back, which she was tolerating well. Her CD4 count was 417 cells μl−1. She presented to us in June 2014 with pain in the right upper quadrant of 3 months duration that was intermittent, colicky, moderate in intensity and radiating to the infrascapular area, suggesting a biliary origin and requiring i.v. analgesics on multiple occasions. She also had low-grade fever, anorexia and weight loss of the same duration. There was no jaundice or other systemic features. Clinical examination revealed a 3-cm firm hepatomegaly; there was no peripheral lymphadenopathy and the rest of the clinical examination was unremarkable. Blood investigations suggested mild anaemia (Hb 10.2 g dl−1), elevated erythrocyte sedimentation rate (47 mm in the firsthour) and deranged liver function tests [serum bilirubin: 0.6 mg dl−1; albumin 4.0 g dl−1; aspartate aminotransferase 49 U l−1; alanine aminotransferase 145 U l−1; alkaline phosphatase 460 U l−1 (normal 42–128 U l−1)]. Ultrasonography of the abdomen showed mild central intrahepatic biliary radical dilatation with a dilated common bile duct (CBD) and multiple periportal and peripancreatic lymph nodes, the largest measuring 10 mm in diameter. Dynamic contrast-enhanced MRI and MR cholangiopancreatography were performed, which revealed a dilated CBD with an abrupt cut-off of the distal CBD with few subcentimetre lymph nodes (). Mantoux test was positive with an induration of 25 × 25 mm at 72 h. Ultrasound-guided FNAC from periportal lymph nodes was performed with a 22-gauge spinal needle and two passes were obtained with the same needle on two separate occasions 1 week apart. However, on cytological examination, the aspirate was bloody and no conclusive diagnosis could be offered by the cytopathologist.
15 days after the second FNAC, the patient developed a painful skin nodule at the site of insertion of the FNAC needle. The nodule measured 1 × 1 cm and was erythematous, well-defined, firm and mildly tender, as shown in (indicated by arrow). FNAC tract seeding by tuberculosis was suspected and excision biopsy of the nodule was performed.
Biopsy of the skin revealed many scattered, ill-formed epithelioid cell granulomas and Langerhans-type giant cells, and periappendageal and perivascular lymphohistiocytic infiltrate admixed with neutrophils and eosinophils. Ziehl–Neelsen stain for acid-fast bacilli was positive (). The overall features were suggestive of tubercular granuloma.
The patient was started on weight-based standard antitubercular therapy with four drugs (isoniazid 200 mg OD, rifampicin 400 mg OD, pyrazinamide 1 g OD and ethambutol 800 mg OD). On follow-up visits, the patient showed good clinical response—fever and pain had subsided, appetite had improved and there was gain in weight. Liver function tests normalized and erythrocyte sedimentation rate decreased to <20 mm in the first hour. A CT scan of the abdomen revealed significant decrease in the size of the periportal and peripancreatic lymph nodes. There was no evidence of biliary dilatation. The patient was doing clinically well after 6 months of antitubercular therapy.
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pmc-6243351-1
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A 32-year-old Mexican immigrant male with no significant past medical history presented to the emergency department with an acute onset of facial tingling and headaches. On physical examination, he was afebrile, normotensive and had no signs to suggest any focal neurological deficits. Basic metabolic panel and complete blood count examinations were within normal limits. Head CT imaging demonstrated a cystic lesion in the right sylvian cistern, suspected to represent an arachnoid cyst (). The patient was discharged after discussion with neurology, with a presumptive diagnosis of migraine with aura.
Subsequently, within 12 hours of discharge, the patient returned with left upper extremity weakness, left facial numbness and speech impairment. Physical examination revealed a left facial droop, left upper extremity weakness and dysarthria. A detailed review of symptoms was otherwise negative. No personal or family history of cerebrovascular events or risk factors was present.
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pmc-6243353-1
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This 66-year-old female was diagnosed with symptomatic carcinoma of the right breast in August 2010. She went on to have a wide local excision and sentinel node biopsy, which was followed by skin-sparing mastectomy and insertion of tissue expander owing to close margins. Histological examination showed a T2N0M0 invasive ductal carcinoma, which was oestrogen receptor positive and HER-2 negative, with no lymphovascular invasion. Her post-operative course was unremarkable and she was started on adjuvant treatment with anastrozole 1 mg daily. In February 2011, she had an exchange of the tissue expander for a permanent fixed-volume textured anatomical cohesive silicone gel implant. This was followed by nipple reconstruction under local anaesthetic in October 2011.
In June 2014, she developed pruritus over the right reconstructed breast and within 3 weeks re-presented with a very enlarged right breast. An ultrasound scan confirmed the presence of a new large seroma, and 600 ml of straw-coloured fluid was aspirated and sent for cytology and microbiology. Following aspiration, it was clinically evident that the implant looked intact, as there was no alteration in its shape. Cytological examination revealed malignancy, showing a population of lymphoid cells () that were positive for CD45, CD30, CD3, CD2 and CD4, and negative for EMA, CD20, CD79a and ALK-1. T-cell receptor gene rearrangement studies confirmed a monoclonal population of T-cells and the diagnosis of BIA-ALCL was established. A contrast-enhanced CT scan of the chest, abdomen and pelvis confirmed an effusion within the right breast implant cavity () and showed no other evidence of disease.
The patient went on to have removal of the implant and complete capsulectomy. There was no evidence of macroscopic rupture of the implant. Histological examination showed focal aggregates of malignant lymphoid cells within the fibrin (capsule) lining the implant cavity. No infiltration outside the cavity was seen. The case was reported to the Medicines and Healthcare Products Regulatory Agency, UK.
A post-operative FDG PET-CT scan showed only low level metabolic activity [maximum standardized uptake (SUV) value of 2.4] that was limited to the chest wall at the recent operative site, which was considered postsurgical (). Based on the clinicopathological assessment, review of the emerging medical literature and imaging results of CT and FDG PET-CT scan confirming no mass lesions, a decision was made to watch and wait. At 3 months, there was no evidence of recurrence on repeat FDG PET-CT scan (). There was again only low level FDG uptake with a maximum SUV of 2.1 and no reaccumulation of the seroma. A 12-month FDG PET-CT scan () showed resolution of the previous FDG uptake, with no evidence of FDG-avid disease.
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pmc-6243356-1
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A 49-year-old female was diagnosed with a pT2N0 (Stage IIA) 2.6 cm invasive ductal carcinoma of the left breast (ER+, PR+, HER-2+, Grade 3/3). She had left mastectomy and 1 month later was booked for a multiple gated acquisition (MUGA) scan to assess her baseline cardiac function before starting adjuvant chemotherapy with trastuzumab, carboplatin and docetaxel. The left anterior oblique (LAO) view images () revealed a small round photopenic defect overlying the septum, which did not change location on the dynamic images. A round focus of absent counts in the region of the septum, measuring approximately 1.5 cm, was also identified on the phase and amplitude parametric images (). The ejection fraction was calculated at 66% and was in the normal range. A chest radiograph revealed a dense ring-like object in the region of the left breast (). 1 month earlier, the patient had a skin-sparing left mastectomy with immediate reconstruction. This was achieved by the placement of a breast tissue expander that was inserted beneath the left pectoralis major muscle (Allergan style 133SV-14-T anatomic saline tissue expander of 375 ml nominal volume). The dense ring-like object seen on chest X-ray was a MAGNA-SITE® (Allergan, Santa Barbara, CA) integrated injection port in the tissue expander, which contains a puncture-proof titanium needle guard and a rare-earth permanent magnet and which is used in conjunction with the MAGNA-FINDER® external locating device (which also contains a rare-earth permanent magnet) for an accurate injection system. The cause of the photopenic artefacts on the MUGA study was the metallic injection port of the left breast tissue expander.
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pmc-6243356-2
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A 41-year-old female was diagnosed with a 0.7 cm invasive ductal carcinoma of the left breast (ER+, PR–, HER2+, Grade 3/3) as well as a right breast 5.5 cm ductal carcinoma in situ (Grade 3/3). She had a skin-sparing bilateral mastectomy with insertion of breast tissue expanders (Allergan style 133MV-14-T 500 ml with an initial fill volume of 250 ml for each side). These expanders also have a MAGNA-SITE integrated injection port in the tissue expander. MUGA scans performed at 2, 5 and 8 months after expander insertion showed a photopenic defect overlying the upper part of the septum (). There was also a semicircular focus of absent counts in the upper part of the septum extending into the left ventricle on the phase and amplitude parametric images (). A chest radiograph revealed two dense ring-like objects in the breast regions (), which were the tissue expander metallic injection ports. The tissue expander design for the two patients is shown in and the metallic injection port in .
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pmc-6243357-1
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A 42-year-old female with a history of systemic lupus erythematosus diagnosed at age 16 years (treated with azathioprine, cyclophosphamide and prednisone) and a renal transplant owing to lupus nephritis at age 30 years (treated with mycophenolate mofetil) presented with sudden onset of confusion and difficulty finding words. Contrast-enhanced MRI of the brain revealed two enhancing brain lesions (), and the patient was referred for 18F-fludeoxyglucose positron emission tomography/CT (18F-FDG PET/CT) imaging, which showed innumerable 18F-FDG-avid lung lesions with a maximum standardized uptake value of 12.1 ( and ), which were confirmed to be Grade 3/3 lymphomatoid granulomatosis (LYG) on lung wedge biopsy () [certain nodules showed >100 Epstein–Barr virus (EBV)-encoded RNA-positive cells per high power field, ]. There was also intense focal 18F-FDG uptake in the distal oesophagus with a maximum standardized uptake value of 8.5, which prompted a gastroscopy and biopsy, revealing an EBV-positive oesophageal ulcer that was treated with long-term valganciclovir 450 mg by mouth daily. She was treated with 4 weekly cycles of rituximab. A follow-up PET/CT scan performed 4 weeks after completion of rituximab showed complete metabolic resolution of LYG lung lesions as well as the EBV oesophageal ulcer (), and a follow-up MRI revealed complete resolution of the brain lesions (). Surveillance PET/CT () and MRI () studies performed 12 months later confirmed disease remission.
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pmc-6243360-1
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A 76-year-old immunocompetent male presented to our institution with a 1-week history of increasing breathlessness and intermittent chest tightness. He was an ex-smoker and his only significant past medical history was deep vein thrombosis and renal stones. On admission, he was found to be bradycardic with a heart rate of 47 beats per minute; clinical examination was otherwise unremarkable. His blood test showed an elevated cardiac troponin-T level of 224 ng ml−1 (reference range <0.01 ng ml–1) and electrocardiogram demonstrated complete heart block with narrow QRS complexes. He was diagnosed and treated as acute coronary syndrome. His chest X-ray was unremarkable and a repeat electrocardiogram the following day demonstrated second-degree atrioventricular block (Mobitz Type 2), but he remained haemodynamically stable. Transthoracic echocardiogram revealed mild hypertrophy of the ventricles with hypokinesis in the lateral wall but preserved left ventricular systolic function. He subsequently underwent successful percutaneous intervention with two drug-eluting stents to a significant stenosis in the circumflex artery. He remained in second-degree atrioventricular block 5 days later, hence a dual-chamber pacemaker was implanted and the patient was discharged home in stable condition.
His general condition deteriorated soon after and he was readmitted within 2 months with worsening breathlessness and chest discomfort. Blood tests showed mild microcytic anaemia. Transthoracic echocardiogram and transoesophageal echocardiogram confirmed marked hypertrophy of both ventricles with granular speckled appearance suspicious of infiltrative disease. In addition, the left atrium was lined with an echogenic speckled mass encroaching on the the mitral valve leaflets (; ). He denied any B symptoms. Myeloma screening was negative and rectal biopsy excluded amyloidosis. CT scan of his thorax confirmed bilateral pleural effusion and an extensive abnormal soft tissue infiltrating the pericardium, much of the myocardium and the inferior portion of the left atrium, extending up to the aortic arch into the superior mediastinum (). Pleural fluid cytology was inconclusive. The patient was commenced on i.v. dexamethasone and rasburicase for suspected lymphoma, with significant improvement in his general condition and resolution of his heart failure symptoms. He subsequently underwent mediastinosopic biopsy of the mass, which confirmed it to be high-grade non-Hodgkin’s lymphoma of B-cell type, with immunocytochemical stain positive for CD45 and CD20. He was commenced on rituximab, gemcitabine, cyclophosphamide, vincristine and prednisolone (R-GCVP), which is the preferred regimen for diffuse large B-cell lymphoma with cardiac involvement. He responded very well to the first two cycles of chemotherapy, achieving remission echocardiographically and radiologically (; ). However, he could not tolerate intensification or extension of treatment beyond six courses. He had multiple hospital admissions during the course owing to neutropenic sepsis requiring antibiotics and intermittent cessation of chemotherapy. In addition, he also developed deep vein thrombosis in his left leg and thrombus attached to the pacing lead that required anticoagulation (; ). A month after completing the chemotherapy, he re-presented with increasing breathlessness, low backache and chest discomfort and a positron emission tomographic scan confirmed a metabolically active mediastinal mass, which was consistent with recurrence (). Although the tumour had originally responded quite well to chemotherapy, it had recurred very quickly and at a very rapid rate. He could not tolerate more intensive chemotherapy, hence palliative oral chemotherapy (DECC regimen) consisting of dexamethasone, etoposide, chlorambucil and lomustine was initiated. Despite initial stabilization, he continued to deteriorate, and a repeat chest X-ray confirmed clear evidence of lymphoma progression (). Chemotherapy was discontinued after discussion with the patient and family and he passed away peacefully at home a week later.
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pmc-6243361-1
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A 19-year-old female presented with sore throat, right-sided jaw pain and a right neck mass that had been present for the past 4 months. A neck ultrasound scan showed a markedly vascular mass in relation to the right carotid sheath. MRI showed an enhancing mass in the right carotid space near the bifurcation, extending superiorly to within 1 cm of the skull base. Characteristic splaying of the carotid artery and internal jugular vein (), as well as typical contrast enhancement with flow voids suggested a vagal paraganglioma (VPG) with a less likely differential diagnosis of schwannoma. There were also multiple enlarged jugular chain and lateral retropharyngeal lymph nodes, which were felt likely to be reactive given the patient’s young age. Urinary catecholamines were in the normal range and MRI of the abdomen demonstrated no adrenal or extra-adrenal PG.
The lesion was approached surgically via a cervical incision and abnormal hypervascular solid lymph nodes were apparent in levels 2 and 3. Frozen section showed paraganglioma cells in the lymph nodes, confirming a malignant tumour. In retrospect, these nodes had similar signals and enhancement as the primary tumour (). Resection of the malignant tumour was performed, which included sacrifice of cranial nerves X and XII. These were reconstructed with ansa cervicalis nerve transfer onto the recurrent laryngeal nerve for vocal cord tone and greater auricular nerve cable graft to the XII defect.
Formal histopathological examination demonstrated a malignant VPG involving 4 out of 13 level 2 and 3 cervical lymph nodes, and involved surgical margins at the skull base. The patient subsequently underwent post-operative radiotherapy and was referred for genetic testing and family counselling.
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pmc-6243361-2
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A 54-year-old male presented with left jaw swelling with a background of a primary left mandibular ameloblastoma resected 4 years ago. CT suggested soft tissue recurrence lateral to the mandible as well as an enlarged left level 1b submandibular node with heterogeneous internal density (). This was felt most likely to be reactive owing to the rarity of nodal metastases in ameloblastoma, and surgical planning was for excision of the local recurrence with primary closure of the neck skin.
The patient underwent excision of the soft tissue mass with en bloc resection of the node. Histological examination showed recurrent ameloblastoma in the soft tissue, with metastatic ameloblastoma within the submandibular lymph node. A second stage selective neck dissection of levels 1–3 was performed and 0/23 nodes contained malignancy. Radiation therapy was discussed with the patient but not undertaken. The patient is undergoing annual surveillance at our centre and has no evidence of recurrence 18 months post revision surgery.
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pmc-6243361-3
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A 72-year-old female presented with right-sided neck swelling 1 year after craniofacial resection of a Hyams grade 2 olfactory neuroblastoma, which initially presented with 6 months of right-sided nasal obstruction, anosmia and facial swelling. The patient received adjuvant radiotherapy to the primary site.
Her initial MRI demonstrated the primary tumour as a large, destructive mass in the right maxillary sinus (). Restaging CT scan of the neck at 12 months showed hyperenhancing cervical lymphadenopathy (). Ultrasound examination showed hypervascular right levels 1B and 2 lymph nodes and was used to guide fine-needle aspiration, confirming metastatic olfactory neuroblastoma.
The patient went on to have a right-sided comprehensive neck dissection followed by radiotherapy to the right neck. Follow-up positron emission tomography (PET) at 3 months demonstrated T10 and left humerus metastases, from which the patient was asymptomatic. Local radiotherapy was provided to the T10 metastasis. However, the bony metastases progressed within the following year and the patient subsequently died.
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pmc-6243362-1
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In September 2013, a 58-year-old female presented to her general practitioner with progressive paraesthesia in her lower limbs. Her past medical history included asthma, well-controlled Type 2 diabetes, and a 6-year history of trigeminal neuralgia and CM1.
A cranial MRI in June 2007 had shown no neurovascular conflict or trigeminal nerve disruption. However, the cerebellar tonsils were positioned 25 mm below the FM, consistent with CM1, in addition to compression of the medulla oblongata and flattening of the anterior surface of the pons. The posterior fossa was mildly underdeveloped but there was no evidence of SHM ().
At presentation in 2013, her facial pain was medically controlled with carbamazepine (400 mg twice daily) but could be triggered with a pinprick in the left maxillary region. The remainder of her neurological examination and nerve conduction studies were normal. Surprisingly, a follow-up MRI showed that the CM1 had resolved ().
The patient’s lower limb paraesthesia resolved spontaneously within a matter of weeks, with no identifiable cause. By August 2015, her trigeminal neuralgia was once again well controlled with carbamazepine (400 mg twice daily), with no episodes of breakthrough pain. At no point was surgery or any other intervention performed.
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pmc-6243364-1
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A 72-year-old male with a long-standing history of polyostotic fibrous dysplasia presented with chest and back pain and was evaluated with a 99mTc-methylene diphosphonate (MDP) bone scan, which revealed intense uptake in several right facial bones, including the frontal, zygomatic and nasal bones, maxilla and the mandible. There was also intense uptake involving the right ribcage (). The patient was referred for 18F-fludeoxyglucose (FDG) positron emission tomography (PET)/CT imaging to evaluate for possible malignant transformation. Maximum intensity projection images showed intense heterogeneous 18F-FDG uptake in the bones of the right face and right hemithorax (). The 18F-FDG uptake in the facial bones was variable, with the maximum standardized uptake value (SUVmax) ranging from 2.1 to a maximum of 5.4 in the right maxillary bone. The right hemithorax lesions appeared to arise from the right eighth and ninth ribs, with involvement of the T7 vertebra, which was collapsed. The SUVmax in the right hemithorax lesions ranged from 4.0 to 7.5 (). Mildly FDG-avid lesions were also noted in T4 (SUV 3.1), left lateral tenth rib (SUV 2.2) and left sacral wing (SUV 2.4). The heterogeneous nature of 18F-FDG uptake and the wide range of SUVmax values raised concern of malignant transformation (or sarcomatous degeneration), and follow-up with a CT scan was recommended. Subsequent 8 years of follow-up with CT scans () did not reveal the development of any aggressive bone lesions, and the patient remains clinically stable with no evidence of malignant transformation.
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pmc-6243369-1
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A-21-year-old-male patient presented with hoarseness of voice and occasional difficulty in breathing. The patient was involved in a road traffic accident 9 months ago and had sustained a head injury. He underwent surgery for depressed fracture of the parietal bone. The patient was managed on endotracheal intubation for 7 days, followed by elective tracheostomy. Later, he was put on T-piece mode of ventilation. Subsequently, he was decannulated successfully after 4 months. 10 days following decannulation, the patient developed change of voice—hoarse in nature and breathy in quality—chronic cough and difficulty in breathing. He was conservatively treated and later referred to our centre for further management. Video laryngoscopy showed a phonatory gap owing to restricted adduction of both vocal cords. Inflammatory markers were negative. Gastrointestinal endoscopy was normal. Multidetector CT (MDCT) scan showed soft tissue density in the cricopharyngeal region, which was encroaching on the tracheal air column from the posterior aspect. The cricoid ring was incomplete, fragmented with sclerotic components within the soft tissue density (). The extent of narrowing of the air column and soft tissue bulge along the posterior wall of the subglottic trachea was well illustrated with a coronal image and a surface-rendered three-dimensional reconstruction (). Voice recording showed severe hoarse voice with pitch breaks and a maximum phonatory duration of 6 s. Finally, based on clinical and imaging information, a diagnosis of cricoid chondronecrosis following prolonged intubation was considered. The patient was managed conservatively with steroids, physiotherapy and nebulization.
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pmc-6243370-1
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A 75-year-old female with well-controlled hypertension presented with a 2-week history of palpitations. On examination, her pulse was regular at 76 bpm, blood pressure was 150/70 mmHg and auscultation revealed a continuous murmur heard over the precordium. She had no signs of heart failure. Resting electrocardiogram (ECG) was within normal limits, and the chest X-ray showed prominent pulmonary arteries on the left side, but normal heart size and clear lung fields. Transthoracic echocardiogram revealed normal left ventricular systolic function with normal ejection fraction at 65%. There was impaired relaxation with Grade 1 diastolic dysfunction. The right ventricular structure and function were normal and there were no significant valvular lesions, with only trivial mitral and tricuspid regurgitation. 24-h Holter monitoring demonstrated a normal heart rate profile, with frequent isolated ventricular and supraventricular ectopic beats, and one episode of broad complex tachycardia of 4 beats. She had blunted heart rate response to exercise and the maximum heart rate was 102 bpm despite completing Stage 3 of the normal Bruce protocol. At peak exercise, the blood pressure dropped from 150/94 mmHg to 90/68 mmHg, and this was associated with presyncope.
Coronary angiography showed a large communication between the left anterior descending (LAD) artery and the main pulmonary artery. In addition, there was another smaller communication that appeared to run from the right coronary artery (RCA) to the pulmonary artery. She was subsequently referred for CT coronary angiography (CTCA), which was performed using a 64-slice multidetector CT scanner using prospective ECG-gated acquisition. This confirmed the presence of a large complex fistula, between the main pulmonary conus and both the LAD and proximal RCA, with cavernous malformation of the fistula segment to the LAD (–). There were two branches of the fistula, the larger branch communicating to the LAD and a further smaller branch to the RCA. The scan also showed minimal plaque burden in the RCA. The patient was reluctant to consider surgical correction of the fistulae, and as she was largely asymptomatic, medical management was pursued. 13 years following the initial diagnosis, she remains asymptomatic with normal left and right ventricular echocardiographic parameters on follow-up.
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pmc-6243371-1
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A 47-year-old female presented to an outside institution with chronic low back pain and right L5 radiculopathy, and an MRI of the lumbar spine was performed (not shown). It showed a mass-like lesion at the right L5–S1 foramen and a diagnosis of hernia or tumour was considered. Over a 1-month period, the patient was managed clinically with analgesics with partial resolution of symptoms.
The patient then presented to the emergency department of our hospital owing to her persistent symptoms and an MRI of the lumbar spine (T1 and T2 weighted sequences) was obtained (). At this point, no contrast injection was performed as it is not included in the low back pain/radiculopathy investigation protocol in the emergency department. It showed the previously described lesion at the right L5–S1 foramen, as well as other ipsilateral lesions in the posterior paraspinal muscles and the anterior epidural space. Most of the lesions had a mass-like appearance and low signal intensity in all MR sequences. Interestingly, one lesion in the posterior paravertebral muscle showed mixed signal intensity on T2 weighted sequence, defining a fluid–fluid level. All the lesions were unchanged compared with the outside scan. The radiology team suspected that the lesions were calcified and suggested a contrast-enhanced CT scan of the lumbar spine ( and ) to confirm their initial suspicion and rule out possible soft tissue components associated with the lesions. The CT scan confirmed the calcified nature of the lesions and also showed no contrast enhancement. It also contributed to an additional finding of interstitial lung disease, as seen in a few images at the base of the lungs in the thoracolumbar region. A CT scan of the chest was suggested () and showed signs of pulmonary fibrosis and oesophageal dilatation. At this time, the hypothesis of paraspinal tumoral calcinosis secondary to SSc was suggested.
Additional clinical investigation showed that the patient had a history of dyspnoea, generalized weakness, arthralgia and gastro-oesophageal reflux disease. On physical examination, Raynaud’s phenomenon with a distal phalanx skin ulcer of the left third digit, sclerodactyly and multiple facial telangiectasias were also observed. Laboratory analysis indicated that creatinine, calcium and phosphorus levels were normal. In fact, the patient had an established diagnosis of lcSSc over a period of 15 years. However, this information was not provided to the radiology department, making the diagnosis of lumbar paraspinal tumoral calcinosis secondary to lcSSc challenging.
After a multidisciplinary team meeting, it was initially decided that the patient’s radiculopathy should be managed clinically with non-steroidal and steroidal anti-inflammatory drugs and follow-up MRI of the spine should be performed within 6 months. In case of non-resolution or worsening of the symptoms during the follow-up interval, it was decided that surgical decompression could be considered depending on the patient’s general clinical status.
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pmc-6243398-1
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A previously healthy 26-year-old Caucasian woman was admitted due to high fever with rash for 2 days. The fever and rash started after lamotrigine was started for her bipolar disorder 1 week ago. However, on further questioning, she also had history of alopecia, arthritis, and oral ulcers intermittently. Her past medical history was unremarkable for rheumatic disease, severe infections, or immunodeficiency. Her family history was also negative for rheumatic disease. On admission, vital signs were normal except for the temperature of 101.5°F. On physical examination, she had diffuse erythematous maculopapular non-itchy rashes over her face and chest without mucocutaneous involvement. Since she complained of the rashes after starting the new medication, we initially treated her as an allergic reaction to the new drug with diphenhydramine and methylprednisolone. However, she continued to have fever spikes along with worsening of her rash.
Laboratory results showed white blood cells 1.7 × 109/L, absolute neutrophils 1.51 × 103/µL, absolute lymphocytes 0.08 × 103/µL, hemoglobin 10.3 g/dL, platelets 138 000 µL, aspartate transaminase 57 U/L, alanine transaminase 19 U/L, triglycerides level 266 mg/dL, fibrinogen 273 mg/dL, ferritin level 16911 ng/mL (normal = 13-150 ng/mL), and elevated lactate dehydrogenase 1767 U/L. Immunological screening was positive for antinuclear antibody (ANA) homogeneous pattern 1250 (normal = 0-49 1/dilution), ANA speckled pattern 6250 (normal = 0-49 1/dilution), anti-double-stranded DNA antibody 344 IU/mL (normal = 0-99 IU/mL), anti-histone antibodies 210 AU/mL (normal = 0-99 AU/mL), serum C3 complement 35 mg/dL, serum C4 9 mg/dL, erythrocyte sedimentation rate 56 mm/h, and C-reactive protein 23.5 mg/L. Blood cultures, urinalysis, and chest X-ray were unremarkable. Viral panel testing for Epstein-Barr virus, cytomegalovirus, herpes virus, hepatitis B and C, HIV, and RPR (rapid plasma reagin) tests were unremarkable. Computed tomography scan of chest showed axillary, cervical, and supraclavicular lymphadenopathy. Since the patient had elevated ferritin and lymphadenopathy seen on computed tomography, there was a concern of MAS. Bone marrow biopsy showed an increased number of macrophages showing hemophagocytosis suggestive of MAS (). Soluble IL-2 receptor was found to be high 3463 U/mL (normal = 223-710 U/mL), and CD 15/56 absolute natural killer (NK) cells was low 7 cells/µL (78-470 cells/µL). We also did axillary lymph node biopsy, which was negative for malignant lymphoproliferative disorder and it showed some hemophagocytosis.
The presence of fever, cytopenia, elevated ferritin, low NK cell activity, elevated soluble IL-2 receptor, and hemophagocytic cells in bone marrow and lymph node led to the main diagnosis of MAS according to the HLH criteria. At the same time, fever, arthritis, oral ulcers, alopecia, high titer ANA, anti-double-stranded DNA, and low complement suggested a diagnosis of SLE according to Systemic Lupus International Collaborating Clinics classification criteria.
The patient was treated with high dose of intravenous (IV) methylprednisolone 125 mg once a day for 3 days. At the end of high-dose methylprednisolone therapy, the patient’s fever resolved and skin rashes disappeared. The ferritin level decreased to 2026 ng/mL. Then, the drug was switched to prednisone 60 mg per os daily and hydroxychloroquine 200 mg per oral twice daily was added. The patient was discharged from the hospital and recommended to follow-up at the outpatient clinic.
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pmc-6243406-1
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A 59-year-old male with a medical history of hypertension presented to the hospital with headache, stiff neck, and nausea. Past medical history included 2 similar presentations: 21 months prior and 1 month prior. On those occasions, cerebrospinal fluid (CSF) analysis showed neutrophilic pleocytosis, and head computed tomography (CT) scan showed a prominent pituitary gland. The patient was treated empirically for bacterial versus viral meningitis on both occasions. Seventeen months prior, the patient was diagnosed with an apparently nonfunctioning pituitary macroadenoma requiring hormone replacement therapy, but surgical resection of the lesion was not pursued. The relevant laboratory values during that episode include an adrenocorticotropic hormone concentration of 11 pg/mL (reference range = 0-46 pg/mL), a thyroid stimulating hormone concentration of <0.01 mU/L (reference range = 0.5-5.0 mU/L), a growth hormone concentration of 0.16 µg/L (reference range = <5 µg/L), and a prolactin level of 42 ng/mL (reference range = <20 ng/mL). The elevated prolactin level was attributed to pituitary stalk compression. The patient was started on levothyroxine 100 µg by mouth once daily, prednisone 5 mg by mouth once daily, and transdermal testosterone gel 5 g to the skin daily.
During all of these encounters, the review of systems was negative for vision loss, rhinorrhea, rash, penile discharge, or recent travel. The physical examination was significant for nuchal rigidity but negative for Kernig’s, Brudzinski’s, or focal neurological deficits.
On presenting for the third time, the patient was again admitted to the hospital for evaluation and management of presumed acute meningitis. Lumbar puncture with CSF analysis showed neutrophilic pleocytosis (see ) with negative bacterial cultures, and negative viral and fungal studies. Magnetic resonance imaging (MRI) of the brain confirmed the presence of a pituitary macroadenoma, which was unchanged from previous imaging. Given the lack of another explanation for the patient’s recurrent meningitis, a transsphenoidal hypophysectomy was performed, and postoperative histopathological examination confirmed the presence of pituitary apoplexy.
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pmc-6243407-1
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A man in his 60s with prostate cancer metastatic to bone and end-stage renal disease was brought to the hospital by his wife due to several days of reduced appetite and inability to ambulate independently. He had a history of strokes suffered 2 years ago and 10 months ago, with residual aphasia. Five months ago, he was admitted for sepsis associated with his tunneled dialysis catheter, with catheter tip and blood cultures having grown Enterobacter cloacae.
On presentation, he was febrile to 104°F, normotensive, had a heart rate of 134 beats per minute, and oxygen saturation was 95%. He possessed a right chest dialysis catheter tunneled to the right internal jugular vein, and a left chest subcutaneous chemotherapy port entering the left subclavian vein. Neurologic examination confirmed expressive aphasia. Antibiotics were started, and he was admitted to the hospital for probable catheter-associated sepsis.
After the patient’s arrival at the medical floor, an intern embarked to obtain differential blood cultures. A large-bore needle was inserted into the chemotherapy port and gentle negative pressure was applied to the syringe, with no return of blood. The attempt was aborted and the intern moved to the right side to inspect the dialysis catheter; however, the patient was found to have lost consciousness. Vital signs were normal, and examination revealed torticollis and gaze deviation to the right; a stroke code was promptly called. After evaluation by the neurologist, the patient was transported for head computed tomography (CT). Approximately 20 minutes later, the patient regained consciousness while on the CT table. The scan showed no acute changes. Ultimately, the event was suspicious more for complex seizure than stroke; therefore, thrombolytic treatment was not given. Nonetheless, follow-up brain magnetic resonance imaging revealed new ischemia in the right and left frontal lobes (). Neurological examination progressed to prominent right-sided weakness, which was not present on admission.
Blood cultures grew Klebsiella pneumoniae. To prevent recurrent bacteremia, the tunneled dialysis catheter was removed at bedside on hospital day 2, and the patient was booked for removal of the chemotherapy port under general anesthesia. He recovered readily from sepsis.
A chart review was conducted. The chemotherapy port had been implanted several years ago and was no longer in use. During the patient’s admission for stroke 10 months ago, contrast echocardiography had revealed a right-to-left shunt, with contrast appearing in the left atrium 2 to 3 beats prior to the right atrium. No atrial septal defect was seen on transesophageal echocardiogram. The technologist noted that the shunt was visible with contrast injected into the left arm, but not the right. This finding was followed-up with a CT venogram of the chest, which failed to identify the source of shunt. At this point, the diagnostic inquiry ended. We sought a radiologist’s review of the CT scan and ascertained that it was nondiagnostic due to incorrect contrast phasing; therefore, we repeated the scan under a pulmonary embolism protocol. This CT angiogram showed left brachiocephalic vein stenosis related to the catheter tip, partial superior vena cava thrombus, and extensive venous collateralization via intercostal, mediastinal, and azygos veins (). There was increased density in the left superior pulmonary vein () suggestive of SPVS. Coronal images revealed filling of the left bronchial venous plexus (), which drains into the pulmonary veins. Interventional radiology was consulted to perform a traditional venogram ().
A risk-benefit analysis was performed, weighing the risk of removing the chemotherapy port to prevent further bacteremia against keeping the port due to the possibility of precipitating further emboli during port extraction. After discussion with the surgery service, it was decided to forgo removal of the port. The patient was bridged with heparin to long-term warfarin therapy. He was referred to the infectious disease clinic for close monitoring, and discharged to subacute rehabilitation.
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pmc-6244219-1
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A fractious 4-month-old female wildebeest (Connochaetes taurinus) weighing 70 kg was presented for surgical fixation of bilateral pelvic limb fractures resulting from a motor vehicle accident (). Anaesthesia was induced with medetomidine (0.01 mg/kg; 40 mg/mL, Kyron Laboratories, South Africa) and ketamine (5 mg/kg; 100 mg/mL, Ketamine Fresenius, Fresenius Kabi, Midrand, South Africa) intramuscularly. The wildebeest was intubated using a 10 millimetre internal diameter PVC endotracheal tube and anaesthesia was maintained with isoflurane (Isofor, Safeline, Weltevreden Park, South Africa) in oxygen (end-tidal concentration 0.8% – 1.1%). Intravenous fluid support was provided in the form of an isotonic crystalloid (lactated Ringers solution, 10 mL/kg/h) and a hydroxyethylated starch colloid (two 10 mL/kg boluses in the first 3 hours of anaesthesia; Voluven; Fresenius Kabi, Midrand). Clinical examination under general anaesthesia revealed a water-hammer pulse, cold extremities and marked pallor. Clinical parameters and arterial blood gas analysis results are shown in . Direct arterial blood pressure was satisfactory 2 h after induction of anaesthesia but decreased thereafter to levels associated with hypoperfusion in mammalian species (mean arterial blood pressure less than 60 mmHg [Dugdale ]) (). Arterial blood gas analysis 2 h post-induction revealed moderate acidaemia, with hypercapnoea, hyperlactataemia, mild hypocalcaemia and moderate hyperkalaemia. Intermittent positive pressure ventilation was initiated using flow-controlled ventilation, with a peak inspiratory pressure of 10 cmH2O, inspiratory to expiratory ratio of 1:2, tidal volume of 500 mL and initial frequency of 20 breaths/minute (SurgiVet Large Animal Ventilator, Smiths Medical, Dublin, OH, United States). The hypercapnoea was successfully corrected and thereafter the respiratory frequency decreased. Calcium borogluconate (10 mL, 40% m/v, Lionel’s Veterinary Supplies, Johannesburg, South Africa) was administered to treat the hypocalcaemia. Dextrose (1 mL/kg 50% solution) was administered to treat the hyperkalaemia. Analgesia was provided in the form of morphine (0.05 mg/kg IV q2h), meloxicam (0.5 mg/kg SC q48h) and a single bolus of ketamine (1 mg/kg IV 3 h after induction, at the start of surgery). The intra-operative haematocrit was 0.13 (2 h after induction); this decreased to 0.12 (3 h post-induction).
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pmc-6244562-1
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A 62-year-old obese lady presented with a 2-month history of dysphagia for solid foods which had worsened over the past 2 weeks, progressing to odynophagia. She was otherwise well and not on any medications. She did not smoke, and only drank moderately.
Oesophago-gastroduodenoscopy (OGD), biopsy and CT revealed a 5-cm-long circumferential, invasive and poorly differentiated adenocarcinoma at the GOJ (Fig. ). Subsequent PET-CT showed no evidence of FDG avid local or distant spread giving a clinical (cTNM) stage of IIA (cT3 N0 Mx) with a mildly avid standardised uptake value (SUV) of 5. Staging laparoscopy confirmed no peritoneal disease, so a feeding jejunostomy was placed. An endoscopic ultrasound (EUS) was not carried out due to the stricturing cancer and as it was thought to have been unlikely to lead to any changes in the treatment plan.
At the time of her initial presentation, our trust protocol for patients with a tumour staged as being T2 N0 or above was neoadjuvant chemotherapy without radiotherapy. She underwent three cycles of epirubicin, cisplatin and capecitabine (ECX) neoadjuvant chemotherapy. Restaging with PET-CT 3 months later showed that the cancer stage remained the same at IIA and that there was a marginal reduction of the SUV to 4.4.
A hybrid Ivor Lewis oesophagectomy with laparoscopic gastric mobilisation was performed with no intra-operative complications. Lymphadenectomy was performed en bloc of stations 4R, 7, 8L, 8M, 9, 10L, 10R, 15–20 (AJCC 7th Esophageal Cancer Staging Manual, 7th Edition). As the specimen was removed through the thoracotomy site, a wound protector was not used. No spillage or perforation of the tumour was noted at time of surgery. The oesophagogastric anastomosis was performed at the level of the thorax, above the azygous vein and 3 cm below the thoracic inlet. Two chest drains were placed as per standard practice. She recovered with no major post-operative complications and was discharged home day 8 post-operative.
The pathological examination demonstrated a ypT4a N2 (3/25) L1 V1 M0 R1 tumour, which was not present in the proximal oesophageal margin block and was therefore at least 5 mm from the proximal surgical resection margin. However, the pathological circumferential resection margin (CRM) was tumour positive (R1), defined as tumour involvement within 1 mm of the surgical resection margin [British Royal College of Pathology ()]. The tumour was also seen to be spreading along the submucosa and subserosa of the stomach. Three of 25 lymph nodes examined were tumour positive. There were no regressive changes from neoadjuvant chemotherapy corresponding to a Mandard tumour regression grade of 5. Histologically, the tumour was a poorly differentiated adenocarcinoma (diffuse type) with scattered signet ring cells and overlying reactive squamous mucosa (Fig. ). Immunohistochemistry showed positive staining with CK7.
Due to the R1 staging, she subsequently underwent adjuvant chemoradiotherapy (45 Gy in 25 fractions with four cycles of carbotaxol), which was standard local practice for a R1 specimen.
Eleven months after the oesophagectomy, our patient re-presented with a non-tender and irreducible mass in the right chest wall at the site of the previous chest drain. There were no further lesions or ascites evident on clinical examination.
CT scans confirmed the presence of a chest wall mass (Fig. ). A PET scan showed a mildly FDG avid recurrence in the right lateral chest wall, with no FDG avid metastases or ascites elsewhere, suggesting that the mass was a single site recurrence (Fig. ). A core biopsy of the mass showed fibro-fatty tissue infiltrated by a diffuse-type adenocarcinoma. Immunohistochemistry showed positive staining for CK7, and showed no evidence of TTF1, CD45, oestrogen or progesterone receptors (Fig. ). The morphology and immunophenotype were consistent with the previous oesophageal primary tumour with both samples showing proliferation of epithelioid cells with moderate eccentric eosinophilic cytoplasm, and some signet ring cells with occasional gland formation in the primary location. Additionally, FDG avidity was similar to the initial oesophageal tumour. Due to these features, the mass was likely to be a recurrence of the original oesophageal cancer. Along with the histological and radiological findings, the patient had no respiratory symptoms, including cough or haemoptysis, making lung cancer unlikely.
Following MDT discussion, it was decided to proceed with surgical resection after considering the disease-free interval (DFI) of 11 months, and that the recurrence appeared to be a solitary oligometastasis which has seeded directly. This was favoured over radical chemoradiotherapy as given the age and life expectancy of our patient, we believed that surgical resection would give her the best chance of long term survival. The resection of the metastasis did not take place as our patient was found to have acute kidney injury (AKI) during the pre-operative assessment. Ultrasound and CT scans demonstrated moderate functional bilateral hydronephrosis (Fig. ) with no evidence of urinary lithiasis or periureteral masses. Double J stents were inserted bilaterally with no complications but did not relieve the hydronephrosis. The planned excision of the chest wall lesion was postponed until bilateral nephrostomies were inserted under radiological guidance and renal function returned to normal.
Six weeks after presenting with a chest wall mass, and after the resolution of the AKI, our patient was restaged with a PET-CT scan. This revealed a focus of increased FDG uptake in the liver and also in an incidental Spigelian hernia (Fig. ). It also showed increasing peritoneal free fluid and an increase in size from 3 to 5 cm of the chest wall lesion. Considering the rapid progression of the recurrent disease, she was managed non-operatively with palliative therapy. She passed away 2 months later.
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pmc-6245524-1
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A 42-year-old woman with history of SLE on hydroxychloroquine, mycophenolate and prednisone, complicated by pancytopenia, presented with severe back pain 30 min after taking oral TMP-SMX 800-160 mg for paronychia. She was found to be febrile to 39.1 °C, hypotensive at 88/63 mmHg, and tachycardic at 107 BPM. Laboratory testing revealed a white blood cell (WBC) count of 8.63 × 103/uL (97.3% neutrophils, 0.1% eosinophils, 0.2% lymphocytes, with a baseline WBC of 2 × 103/uL), lactate 2.3 mmol/L and creatinine of 1.3 mg/dL (baseline 0.7 mg/dL) (Table ). HIV ELISA was negative but the CD4+ count was low at 64 cells/uL. Computed tomography (CT) angiogram of the chest showed no evidence of pulmonary embolus or infection and urinalysis was not suggestive of infection. Physical exam was significant for diffusely erythematous and warm skin without macules, papules, or urticaria. There were no other focal findings on physical exam. The patient was initially managed with aggressive 80 mL/kg intravenous (IV) fluid resuscitation, norepinephrine, broad-spectrum antibiotics (IV vancomycin and IV piperacillin-tazobactam), and stress dose steroids (hydrocortisone 50 mg IV every 6 h). Her hypotension resolved quickly over the first 36 h of admission. Over the following 3 days blood and urine cultures remained negative and steroids, vasopressors and antibiotics were discontinued without recurrence of hypotension. Additional infectious work up, including hepatitis B, hepatitis C and toxoplasma, were negative. The patient was discharged home on hospital day four with pneumocystis jirovecii pneumonia (PJP) prophylaxis with atovaquone.
Ten months prior, the patient had a similar presentation several hours after taking her second dose of oral TMP-SMX 800-160 mg, which was prescribed for an abscess of the mons pubis. On presentation, she was hypotensive, tachycardic and febrile. Laboratory testing revealed a WBC of 10.9 × 103/uL (95.5% neutrophils, 0.6% lymphocytes and 0.5% eosinophils), acute kidney injury (creatinine 1.1 mg/dL) and lactate of 1.2 mmol/L. She was treated similarly for presumed septic shock. Work-up for infectious etiologies including blood and urine cultures, CT imaging of head, spine and abdomen as well as an echocardiogram was unrevealing. She was weaned off vasopressor support within 24 h. Her steroids and antibiotics were discontinued within 72 h with clinical improvement and she was discharged home with the diagnosis of septic shock with unknown etiology.
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pmc-6245536-1
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An 86-year-old woman visited our hospital with foamy urine and foul odor. Urinalysis showed many WBCs (163.7 WBCs/μL) and bacteria (11,343.7 bacteria/uL), and positivity for nitrite. Gram-negative coccobacilli were revealed upon microscopic examination. The sample was cultured on sheep blood agar plate (BAP) and MacConkey agar plates at 35 °C in a 5% CO2 atmosphere for 24 h. After one day of incubation, > 100,000 CFU/ml of pinpoint Gram-negative colonies grew on the BAP with 10,000 CFU/ml of Gram-positive cocci. After isolation of pinpoint colonies and another 24-h incubation, the pinpoint Gram-negative colonies were irregularly divided into large colonies and pinpoint SCV colonies on BAP (Table ).
While the VITEK 2 system (bioMerieux, Durham, USA) identified the pinpoint colony as Burkholderia cepacia group, the Bruker Biotyper (Bruker Daltonics, Leipzig, Germany) and VITEK MS (bioMerieux, Marcy-l’Étoile, France) matrix-assisted laser desorption/ionization time-of-flight mass spectrometry (MALDI-TOF MS) systems identified both colonies as E. coli. The 16 s rRNA sequencing concluded both isolates were E. coli. As automated systems in an ambient air were unable to grow capnophilic SCVs, antimicrobial susceptibility testing profile was determined through disk diffusion method []. With the exception of levofloxacin resistance, bacteria was susceptible to all other antimicrobials. From these findings, we concluded that this isolate was CO2-dependent and had the ability to revert to its natural large form in the presence of CO2.
Whole genome sequencing analysis by the MiSeq® system (Illumina, San Diego, USA) was performed to inspect assumed genes that contained previously-reported causative mutations for the E. coli SCV phenotype (hemB, menC, and lipA gene) [, ], but no genetic mutational variations were observed between the two strains. The yadF gene was not present in either strain, which is consistent with previous reports about capnophilic E. coli strains [].
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pmc-6245604-1
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Patient: A 49-year-old male.
Chief complaints: Epistaxis and pain in the left eye.
Past medical history: Colonic polyps.
Life history: Smoking history: 30 cigarettes/d × 20 y (quit smoking at age 39); Alcohol consumption: 350 ml of beer/d.
Allergy history: Unremarkable.
Family history: Unremarkable.
History of the present illness:
The man developed epistaxis and pain in the left eye starting in December 20XX, and he was seen by a nearby physician in January 20XX + 1. A tumor was noted in the left nasal cavity, and needle aspiration cytology was performed. The tumor was initially diagnosed as class V (round cells suggesting malignant lymphoma), so the man’s previous physician referred him to Otolaryngology on January 18. CT and MRI revealed a mass and bone destruction in the left maxillary sinus, the left ethmoid sinus, the left frontal sinus, and the right frontal sinus. On January 21, the man was referred to Otolaryngology at this Hospital. The left nasal cavity tumor was biopsied (Fig. ). Based on a histopathological examination, the man was diagnosed with an ESFT, and he was referred to Internal Medicine on February 1. Laboratory investigations revealed normal levels of the tumor markers SCC (1.2 ng/ml reference range 0–2.5 U/L), and soluble IL2 receptor (434 U/ml reference range 145–519 U/L) (Table. 1). In this hospital, contrast-enhances MRI and PET/CT reveals a mass invading the left maxillary sinus and the left frontal sinus (Figs. and ). A contrast-enhanced nodule in the left ilium was considered as bone metastasis.
Height of 173. 3 cm, weight of 81. 4 kg, body temperature of 36. 8 °C, heart rate of 68 beats/min (regular), blood pressure of 122/65 mmHg, SpO2 of 98% (room air), and alert.
Mild reddening of the left cheek was present. Blepharoptosis of the left eye was present. The left eye was pushed upward and protruded forward. Pupils were 3.0/3.0 mm in size and the pupillary light reflex was absent in the left eye. There were no visual field defects. The eye was in primary position. The left eye was unable to adduct, diplopia was absent, and sensory loss in V1 was present on the left. There was no assymetry of the orbicularis oris. Closing the left eye was difficult. Wrinkling of the forehead was not possible. Dysarthria was absent and a curtain sign was present (the uvula deviated to the right).
Biopsy specimen of tumor tissue revealed a proliferation of undifferentiated round cells that were CD99-positive on immunohistochemistry, so the patient was diagnosed initially with an ESFT (Fig. a, b and c). A vincristine, cyclophosphamide, and doxorubicin (VDC) regimen (vincristine at a dose of 1. 5 mg/m2 on day 1, cyclophosphamide at a dose of 1200 mg/m2 on day 1, and doxorubicin at a dose of 75 mg/m2 on day 1, q3w) for ESFT with distant metastasis was started on February 5 []. Results of a chromosomal analysis (Fig. ) were received during the first course therapy. Instead of revealing chromosome 22 abnormalities as are characteristic of an ESFT, results revealed a translocation involving the long arm of chromosome 15 and the short arm of chromosome 19. Additional immunostaining was positive for anti-NUT antibody positive (Fig. d). Ultimately, the patient was definitively diagnosed with NC. After the conclusion of the first course of therapy, MRI revealed that the tumor had shrunk. Macroscopically, swelling of the left cheek had subsided. A study reported that administering a therapeutic regimen for ESFT prolonged survival in patients with NC [], so the same strategy was continued in the current case. In total, 5 courses of therapy were administered, and the primary cancer shrank. PET/CT revealed that abnormal accumulation of contrast agent in bone metastases disappeared (Fig. ), so Otolaryngology was consulted, and the decision was made to perform surgery for local control. Surgery was to be performed for local control after the sixth course of therapy (ADR was left out in light of its cardiotoxicity), but abrupt swelling of the forehead was noted after the conclusion of the sixth course of therapy. The primary cancer was deemed to have progressed. Circumstances required radical excision in the form of resection of the dura mater and skull base reconstruction, but abruptly modifying that surgery would have been difficult. Given the speed of tumor enlargement, radical excision was not indicated. A strategy to treat locally advanced cancer of the head and neck through standard cytoreduction plus radical chemotherapy and radiation therapy (cisplatin at a dose of 100 mg/m2 on day 1, q3w, radiation of 70 Gy/35 Fr) was adopted, and total excision of the left ethmoid sinus+partial excision of the maxillary sinus were performed on June 21. The lesion was removed to the extent possible, but the tumor had invaded the ethmoid sinus, so the tumor remained. Postoperative histopathology also indicated that most of the tumor tissue consisted of viable cells. On June 27, chemotherapy including cisplatin and radiation therapy were started, and the tumor tended to shrink. Starting on about August 1 (with irradiation of about 50 Gy), however, swelling of the left forehead and the right neck was noted. MRI revealed enlargement of the residual lesion and new lymph node metastases. Radiation was discontinued, and various forms of chemotherapy (therapy with ifosfamide/etoposide, therapy with gemcitabine/docetaxel, and pazopanib) were subsequently initiated, but the tumor grew rapidly, and the patient passed away in October 20XX + 1, 9 months after being diagnosed.
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pmc-6245610-1
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A 70-year-old female had been symptomatic with a persistent non-productive cough and recurrent chest infections for 10 years. At presentation to clinic a CT thorax showed right middle lobe bronchiectasis. She was a life-long non-smoker with no childhood history of respiratory disease and normal baseline investigations for immune deficiency. Clinical stability was achieved with long term Azithromycin and regular airway clearance. Staphyloccocus aureus (S.aureus) was cultured intermittently from sputum samples with repeatedly negative mycobacterial cultures. A repeat CT 6 years after her initial scan demonstrated progression of disease with bi-apical scarring, right middle lobe atelectasis, right upper lobe cylindrical bronchiectasis and reticulo-nodular densities in both lower lobes. (Fig. ) Nebulised Tobramycin was trialled to suppress S.aureus and stabilise radiological appearances but stopped after 15 months due to worsening cough.
M. abscessus was first isolated from her sputum in November 2013; sub-speciated into M.a. abscessus. Commencement of therapy was under consideration when, 3 months from first isolation of M.abscessus, paraesthesia developed in her hands and feet with associated small joint arthropathy. A subtle purpuric rash was evident on the lower limbs. Nerve conduction studies confirmed an axonal loss sensorimotor neuropathy. Erythrocyte Sedimentation Ratio was elevated at 49 mm/hr, Perinuclear Anti-Neutrophil Cytoplasmic Antibodies (P-ANCA) titre was highly positive at 80 AI and Myeloperoxidase- ANCA (MPO-ANCA) was > 8 AI with Proteinase-3–ANCA (PR3-ANCA) < 0.2 AI. Renal function and urine microscopy were normal. A diagnosis of microscopic polyangiitis was made. Treatment with Cyclophosphamide and high dose Prednisolone was commenced with full resolution of her paraesthesia.
Within 4 months of first isolation, all sputum samples (n = 4) were culture positive and 66% of those samples were smear positive for M. abscessus. C-Reactive Protein was elevated at 86.2 mg/l. A repeat CT thorax showed extensive bronchiectasis with underlying collapse in the right upper and lower lobes. There was extensive tree-in-bud change within the right lower lobe, scattered pulmonary nodules and small areas of ground glass shadowing in both lungs. Deteriorating radiological appearances and commencement of active immunosuppression prompted urgent initiation of M. abscessus treatment.
Induction phase treatment comprised intravenous (IV) Cefoxitin and Amikacin with oral Clarithromycin and Minocycline and was tolerated for 2 weeks. Long-term nebulised Amikacin was commenced, along with oral Moxifloxacin, Minocycline and Clarithromycin.
A month later she attended the Emergency Department (ED) with rupture of her right Achilles tendon secondary to Moxifloxacin. This was stopped immediately and replaced with Linezolid. Despite dose reduction this was discontinued due to intolerable nausea, diarrhoea and angular stomatitis. She remained on oral Minocycline, Clarithromycin and nebulised Amikacin. Cyclophosphamide was changed to Azathioprine as long-term immunosuppression, but this was poorly tolerated due to nausea. Significant alopecia then developed; attributed to Minocycline, which was also discontinued. Nebulised Meropenem was therefore added to maintain triple antibiotic therapy.
On review 17 months after initiation of treatment her pulmonary disease appeared more stable. A regime of nebulised Meropenem, nebulised Amikacin and oral Clarithromycin was tolerated. Smear and culture negativity was maintained for a further 12 months on this regime. P-ANCA and MPO-ANCA titres remained within normal limits during this period.
Twenty months after starting treatment, cultures again became positive for M.abscessus. A month after culture positivity returned, she presented to the ED with visual loss secondary to a right branch retinal vein occlusion. MPO-ANCA had again become highly positive at > 8 AI and P-ANCA titre was 160 AI. Due to active vasculitis with impending visual loss from associated hypercoagulability and thromboembolism higher dose immunosuppression was required. She commenced Rituximab and Methylprednisolone infusions weekly for 4 weeks, followed by 6 monthly Rituximab. Clinical response was achieved with CD19 count reducing from 46% to 0%. Within the following 12 months sputum samples became smear positive again. High resolution CT chest demonstrated extensive airspace opacification, ill-defined nodularity and tree in bud change. (Fig. )
Further assessment of immunological function was carried out, including vaccination response, respiratory oxidative burst, Mannan Binding Lectin, IgG subsets, complement levels, alternative and innate signalling pathways. Specific testing of interferon pathways demonstrated a very low interferon-gamma (IFN-γ) level, with low production of Interleukin-17 (IL-17) but no autoantibodies to IFN-γ.
No other organisms, including S.aureus have been detected by standard sputum culture since 2012. Four years from first isolation she remained M.abscessus smear and culture positive. Clarithromycin resistance was now detectable necessitating its cessation. A further 3 weeks of IV Amikacin, Cefoxitin and Tigecycline was commenced. Long-term treatment continued with nebulised Meropenem and Amikacin.
A third oral agent would optimise long-term therapy but intolerances, resistance and risk of visual loss prevent the use of almost all suitable oral agents. Clofazimine was not commenced due to risk of further visual loss. A trial of oral Co-trimoxazole [] resulted in intolerable nausea and had to be stopped. As no third oral agent could be used, pulsed IV antibiotics have been introduced. The optimal duration between IV courses is unknown and is currently based on recurrence of smear positivity or deteriorating symptoms. The first repeat IV antibiotic course maintained smear negativity for 6 months. Recurrence of smear positivity and associated clinical deterioration have required further IV courses (Table ).
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pmc-6245622-1
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A 52-year-old woman presented to our Endocrinology Unit with a growing thyroid mass, which had enlarged so rapidly she had become unable to wear her motorcycle helmet in the weeks prior to her visit. She suffered from Hashimoto’s thyroiditis for which she was taking levothyroxine. There was no history of neck irradiation or family history of thyroid cancer. On examination, there was a large, firm thyroid nodule on the right side of the neck, without palpable cervical lymphadenopathy. TSH was 4.79 μU/mL with FT3 and FT4 within the reference range. Otherwise, there was only a mild thrombocytopenia. Thyroid ultrasonography showed a solid hypoechoic nodule in the right lobe of the gland, with significant internal vascularity and absence of calcifications (Figure ). FNA cytology with rapid on-site evaluation of the material adequacy showed that there were only atypical lymphoid cells with no thyrocytes and the specimens were considered suggestive of a lymphoproliferative disorder but insufficient to make a diagnosis, such that a CNB was scheduled for the following day.
After checking the blood coagulation profile, the patient underwent a CNB, which allowed histological/morphological tissue analysis. This showed that normal thyrocytes were virtually all replaced by homogeneous medium-sized lymphocytes with scanty blue cytoplasm, round nuclei, coarse chromatin, and multiple small nucleoli. There were frequent mitotic figures and scattered macrophages ingesting apoptotic cells, giving to the tissue section the so-called ‘starry sky’ appearance (Fig. ). Overall, these features were consistent with the presence of a thyroid Burkitt’s lymphoma, and further investigations were ordered to confirm the diagnosis and evaluate the disease extent. A CT of chest and abdomen showed the 44x43x87 mm thyroid nodule with left tracheal deviation (Figure ) without other visible masses or lymph nodes. Bone marrow biopsy showed almost 100% lymphoid infiltration, consisting of a population of intermediate-sized blast-like cells, with prominent nucleoli, which were replacing all normal cells. These cells expressed CD10, CD20, and were negative for Bcl2, CD34, and TdT. Altogether these results led us to the final diagnosis of stage IV Burkitt’s lymphoma [].
The patient was admitted to our hospital’s Haematology Unit and was successfully treated with 3 cycles of Hyper-CVAD chemotherapy (cyclophosphamide, vincristine, doxorubicin and dexamethasone) completed in five months. The thyroid mass disappeared (Fig. ) and the platelets returned to baseline levels. At 60 months after diagnosis the patient is alive, and remains disease-free at regular follow-up.
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pmc-6245632-1
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A 76-year-old man with a history of hypertension and benign prostatic hyperplasia consulted a dermatologist with a complaint of pruritus in the perianal area. The doctor diagnosed this area as eczema, and had prescribed Corticosteroid ointment for him for 10 months. Because his symptom did not improve, he received a colonoscopy to check for colorectal malignancy. However, no anal canal lesion was noticed at that time. Two months later, multiple liver lesions were incidentally found during follow-up ultrasonography for his prostatic hyperplasia. Computed tomography (CT) scan revealed multiple liver lesions (Fig. a) and right inguinal lymph node swelling. Pathological examination of biopsies obtained from the perianal erythema showed infiltrating Pagetoid cells and poorly differentiated adenocarcinoma (Fig. a). Immunohistochemistry (IHC) demonstrated malignant cells positive for cytokeratin (CK)-7 and − 20 (Fig. b and c) and negative for gross cystic disease fluid protein-15 (GCDFP-15) (Fig. d). These findings suggested secondary EPD. The lymph node was also pathologically diagnosed as a metastasis. He was referred to our hospital for further examination and treatment.
On admission, his European Cooperative Oncology Group performance status was 0. Physical examination revealed hepatomegaly and erythematous perianal skin lesion (Fig. a). Elastic hard tumor in the anal canal was palpable by digital examination. Serum carcinoembryonic antigen (CEA), neuron specific γ- enolase (NSE), and lactate dehydrogenase (LDH) levels were high, with 809.4 ng/mL (normal range, 0 to 5 ng/mL), 85.8 ng/mL (normal range, 0 to 16.3 ng/mL), and 1176 U/L (normal range, 115 to 245 U/L), respectively. Carbohydrate antigen 19–9 level was normal. Endoscopy showed an elevated tumor of the anal canal like a submucosal tumor (Fig. a and b). Pathological examination revealed poorly differentiated adenocarcinoma (Fig. e) with neuroendocrine features of positive synaptophysin and chromogranin-A expressions (Fig. f and g). Ki-67 showed a high proliferation index of 60% (Fig. h). A KRAS mutation at codon 12 was detected in the primary anal canal lesion. The previously diagnosed perianal skin lesion and lymph node tumor showed the same pathological features. Finally, the patient was diagnosed with metastatic anal canal adenocarcinoma with neuroendocrine features, accompanying secondary EPD.
He received chemotherapy with mFOLFOX6 (oxaliplatin 85 mg/m2, bolus 5-FU 400 mg/m2, and folinic acid 200 mg/m2 on day 1 with 46-h infusional 5-FU 2400 mg/m2, every 2 weeks). Soon after treatment, his hepatomegaly improved day by day. CT scan after 4 courses of mFOLFOX6 showed remarkable tumor shrinkage and morphological changes to homogenous nonenhanced lesions (Fig. b), and the EPD disappeared (Fig. b). Serum levels of LDH, CEA, and NSE decreased promptly to within normal range (Fig. ). The primary anal canal lesion also responded to the treatment (Fig. c and d). The treatment regimen of mFOLFOX6 was switched to CAPOX (capecitabine 2000 mg/m2/day for 14 days and oxaliplatin 130 mg/m2 on day 1, every 3 weeks) due to thrombus formation around the central venous catheter. Currently, he is receiving capecitabine plus bevacizumab together with edoxaban to prevent secondary deep venous thrombosis after removal of central venous catheter as a maintenance therapy, and a good partial response with normal serum tumor markers has been maintained for more than 11 months after the initial treatment.
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pmc-6245707-1
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A 56-year-old woman was introduced to Kochi Medical School from a private hospital for right renal tumor detected by abdominal computed tomography (CT). She had been undergone radical nephrectomy for left renal cell carcinoma (RCC) 7 years before. An abdominal CT of the present tumor revealed a right renal tumor, 5.3 cm in diameter, showing poorly-defined margins, irregular contrast and no findings of metastases (Fig. , ). An abdominal CT that was performed 7 years ago revealed a left renal tumor, 7.0 cm in diameter, showing well-defined margins, irregular contrast and no findings of metastases, diagnosed clinical stage T1b N0 M0 left RCC (Fig. , ). She did not have any other medical history or family history.
Open right partial nephrectomy was performed under a presumed diagnosis of clinical stage T1b N0 M0 right RCC, recurrent or due to metastasis from the previous left tumor. The tumor was a macroscopically well-circumscribed solid mass. The cross-sectional surface was lobulated and heterogenously yellow to brown with bleeding and necrosis (Fig. ). Microscopically, the tumor showed an alveolar growth pattern admixed with eosinophilic and clear cytoplasm. Papillary architecture was also focally seen. In some areas, eosinophilic coarse granules were identified in the tumor cytoplasm. Pathological stage was pT1b pN0 with negative surgical margin. Nuclear Grade corresponded to largely Fuhrman Grade 3 and partly Grade 4. Hyaline nodules and psammoma bodies were observed in the stroma. Immunohistochemically, the tumor cells showed diffuse positivity for renal cell carcinoma-maker (RCCMa, PN-15, 1: 100, Cell Marque, CA, USA) and cluster differentiation (CD)10 (56C16, prediluted, Novocastra Laboratories Ltd., Newcastle, UK) and negativity for Cathepsin K (3F9, Abcam, Tokyo, JP), Melanosome (Human melanoma black; HMB45, prediluted, DAKO, Glostrup, Denmark), Melan A (A103, 1: 100, Novocastra Laboratories Ltd., Newcastle, UK), and alpha smooth muscle actin (data not shown). Seventy percent of neoplastic cell nuclei stained positive for TFE3 (MRQ-37, prediluted, Ventana Medical Systems, Inc., Tucson, AZ), with a staining intensity of (moderate) 2+ to (strong) 3+ (Fig. ). Staining for transcription factor EB (TFEB, polyclonal, V-17, 1: 400, Santa Cruz, Biotechnology, Inc., Dallas, TX) was generally negative (data not shown).
Hematoxylin and eosin, and immunohistochemical stains from the previous tumor were retrospectively reviewed. In H and E staining, tubular, papillary, and alveolar growth patterns were noted admixed with eosinophilic and clear cytoplasm. Additionally, very large tumor cells were seen and dedifferentiation with a discohesive area and rhabdoid features was also noted. Necrosis and hemorrhage were present. Pathological stage was pT1b pN0. Nuclear Grade corresponded to Fuhrman Grade 4. Small venous invasion by carcinoma cells was seen. Neoplastic cells showed diffuse immunohistochemical expression of RCCMa, CD10, Alpha-Methylacyl-CoA Race (AMACR; P504S, 13H4, 1: 100, DAKO, Glostrup, Denmark) and negative results for cytokeratin 7, Carbonic Anhydrase IX (CA9, D47G3, Cell Signaling, MA, USA), HMB45, Melan A and Cathepsin K (data not shown). TFE3 was positively stained in the nuclei of 5% of neoplastic cells with a staining intensity of 2+ to 3+ (Fig. ).
We performed a dual-color, break-apart fluorescence in situ hybridization (FISH) assay to identify the chromosomal break point of TFE3 in paraffin-embedded tissue []. Briefly, the break-apart FISH assay with probes upstream and downstream to TFE3 showed red and green signals. A fused or closely approximated green-red signal pattern was interpreted as a normal result, whereas a TFE3 fusion resulted in a split-signal pattern. Signals were considered to be split when the green and red signals were separated by a distance of more than 2 signal diameters. For each tumor, a minimum of 100 tumor cell nuclei were examined under fluorescence microscopy at × 1000 magnification. Only nonoverlapping tumor nuclei were evaluated. Positive findings were defined as more than 10% of the tumor nuclei showing the split-signal pattern []. The TFE3 gene showed gene splitting in 71.55% of 130 neoplastic cells and in 76.82% of 233 neoplastic cells in the present and the previous tumor, respectively. Typical TFE3 break-apart signals of the present and previous tumors are presented in Fig. .
Total RNA was extracted from formalin fixed paraffin embedded tissue of the previous tumor and from frozen tissue of the present tumor using a standard organic extraction method (MACHEREY-NAGEL, Germany and QIAGEN, Germany, respectively). ASPL-TFE3 fusion transcripts were detected using an ASPL forward primer: 5’-AAAGAAGTCCAAGTCGGGCCA-3′ and a TFE3 exon 4 reverse primer: 5’-CGTTTGATGTTGGGCAGCTCA-3′. Glyceraldehyde-3-phosphate dehydrogenase (GAPDH) transcripts were detected using the forward: 5’-CGGATTTGGTCGTATTGG-3′ and reverse: 5’-TCCTGGAAGATGGTGATG-3’ GAPDH primers []. The ASPL-TFE3 fusion gene was detected in the tissue from the present and the previous tumor but was not detected in the normal tissue. GAPDH that was used as a loading control was detected in each reaction (Fig. ).
There is a no evidence of recurrence at 8 months postoperatively.
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pmc-6245774-1
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A 22-year-old woman, gravida 4, para 2, one early abortion and 2 alive children, was transferred from a medical center to the maternity ward of the University Teaching Hospital of Ouahigouya (Burkina Faso) for bowel sub-obstruction and intrauterine fetal death, with failure of labor induction, on an assumed full term pregnancy.
She first consulted for moderate abdominal pain that had been going on for 10 days, at the health center in her village, where early childbirth labor was diagnosed. The day after she arrived, she was evacuated to the referral medical center for fetal distress suggested by an abnormal decreasing of the fetal heart rate.
The history of the pregnancy notes that the patient, who did not know the exact date of her last menstrual period, had done in the health center of her village, 05 antenatal consultations during which no particular anomaly was noticed. The symphysio-fundal height grew steadily up to 30 cm at the last visit, with a presumed cephalic presentation. The patient did not perform any ultrasound or other blood tests outside the HIV serology that was negative. She did not experience pelvic pain or metrorrhagia at the beginning of her pregnancy, and never consulted for any pathology during her pregnancy. She had no particular medical and surgical history.
On admission at the maternity ward of the University Teaching Hospital of Ouahigouya, the patient no longer complained of abdominal pain, but reported respiratory discomfort due to abdominal distension, and absent fetal movements.
She had normal hemodynamic state, but mild pallor. Her abdomen was distended, and the fetal parts were palpated under the maternal abdominal wall, with difficulty in specifying the presentation. The sounds of the fetal heart were not perceived. At the vaginal touch, the cervix was anterior, short, soft, and dehiscent, and the fingerstall was stained with traces of blood. There was no ileus.
Diagnostic hypothesis of uterine rupture or abdominal pregnancy with intrauterine fetal death were thought about.
Emergency ultrasound scan noted an empty uterus of subnormal size, a fetal death and a lateral uterine mass reminding the placenta.
An emergency laparotomy was then indicated for suspicion of abdominal pregnancy or uterine rupture. At the opening of the peritoneum, a bloody fluid (most likely blood, amniotic fluid and peritoneal fluid) was sucked up. The fetus that bathed in the abdominal cavity was extracted dead and macerated (Fig. ). It weighed 3300 g and did not have any malformation. The placenta was fully inserted into the hypertrophied and ruptured isthmus of the left fallopian tube (Fig. ). The ipsilateral ampullary and infundibular portions were free and normal-looking (Fig. ). The uterus was non gravid and had normal size. The abdominal viscera were free from adherence. The diagnosis of full term abdominal pregnancy, with implantation of the placenta into the isthmus of the left tube was then retained. A left total salpingectomy removing in the same time the placenta (Fig. ) was performed. The patient received a blood transfusion, and the postoperative follow-up was simple.
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pmc-6245778-1
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Patient 1 was a 4-year-old male. The onset of the disease was 20 months before the transplantation. He presented with symmetrical proximal muscle weakness, dyspnea, dysphagia and dysphonia. On physical examination, his muscle strength was as following: right lower extremity proximal 1–2/5, distal 3/5; left lower extremity proximal 3/5, distal 4/5; upper extremities proximal 3/5, distal 4/5. He had positive Gottron’s sign and heliotrope rash. His laboratory results showed: CK 1200 U/L (0–195 U/L); negative antinuclear antibody (ANA). Electromyogram (EMG) showed myogenic damage. Muscle MRI showed diffuse muscle involvement of proximal legs. HRCT showed mild pulmonary interstitial disease. A left quadriceps biopsy showed extensive muscle atrophy, focal necrosis, small vessel wall degeneration and thickening, fibrous thrombosis, and fatty tissue hyperplasia. Initially we gave him intravenous immunoglobulin (IVIG) (2.0 g/kg per month for 3 months), cyclophosphamide (CTX) (1 g/m2 body surface area monthly intravenously for 6 months), and high-dose methylprednisolone (MP) (20 mg/kg per day for 3 days) followed with prednisone 2 mg/kg daily. After 6 months of treatment, his rash, expiratory dyspnea, dysphagia and dysphonia improved, but muscle weakness remained. Therefore, we recommended AHSCT and he received the transplantation in June 2005.
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pmc-6245778-2
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Patient 2 was a 7-year-old girl. The onset of the disease was 31 months before AHSCT. Her symptoms included fever, muscle weakness, dysphonia, dyspnea and dysphagia. On physical examination, her muscle power was as following: right lower extremities proximal 2/5, distal 4/5; upper extremities proximal 3/5, distal 3/5. Gottron’s sign is positive. Her laboratory results showed serum CK 500 U/L (0–195 U/L) and negative ANA. EMG showed myogenic damage. Muscle MRI showed diffuse muscle enhancement of proximal legs and limbs. HRCT showed severe pulmonary parenchyma and interstitial disease. A right quadriceps biopsy showed denatured, broken and dissolved muscle, along with focal chronic inflammatory cells and positive Masson staining. We intubated her and placed her on a ventilator, and simultaneously gave her IVIG (2.0 g/kg per month•3 months), CTX (1 g/m2 body surface area intravenously monthly for 6 months), high-dose MP (20 mg/kg daily for 5 days) and followed by prednisone 2 mg/kg daily. Two weeks later, her dyspnea improved, and tracheal intubation was removed. One month later, her dysphagia and dysphonia improved. But the improvement of muscle weakness and rash was not obvious. So we gave her methotrexate (MTX) 15 mg/ m2 body surface area and cyclosporine A (CsA). Nine months after the initial treatment, her muscle weakness and rash were not improved. Therefore, we recommended AHCST and she received the transplant in January 2008.
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pmc-6245778-3
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Patient 3 was a two and half years old female. The onset of JDM was 6 months before the transplant. She presented with muscle weakness and dysphagia. On physical examination, her muscle strength was as following: lower extremities proximal 2/5, distal 3/5; upper extremities proximal 2/5, distal 3/5. Gottron’s sign was positive. Her laboratory tests showed high serum CK 2569 U/L (normal: 0–195 U/L) and negative antinuclear antibody. EMG showed myogenic damage. Muscle MRI showed diffuse muscle involvement of proximal legs. HRCT showed spot shadow in left lung, and focal interlobular septal thickening. Parents refused muscle biopsy. Initially, we gave her IVIG (2.0 g/kg per month•3 months), CTX (1 g/m2 body surface area intravenously monthly for 5 months), high-dose MP (20 mg/kg daily • 5 days), followed by prednisone 2 mg/kg daily. After 5 months of treatment, her rash and dysphagia improved, but muscle weakness remained the same. Therefore, we recommended AHCST, and the girl received the transplant in July 2015.
Three to eight days after the AHSCT, the leukocyte and lymphocyte levels of all 3 patients decreased to the lowest level (0.01 × 109/L). The platelets decreased to 5–10 × 109/L. Haemoglobin (HGB) decreased to 30–60 g/L. 10 to 14 days after AHSCT, the neutrophils increased to more than 1.0 × 109/L. 14 to 16 days after the AHSCT, the platelets came back to 20 × 109/L. Those results indicated that AHSCT for all three patients were succeeded.
The immunological function was obviously inhibited after the auto-PBHSCT (Fig. ). The number of CD4 and CD8 cells remained low within 3 months after the transplantation. 6 months later, the number of CD4+ and CD8+ cells returned to normal.
In the first 6 months after AHSCT, muscle weakness and rash improved slowly for all three patients. However, after 6 months, the improvement was much faster. About 12 months later, all of their monitoring indexes, including immunological function, CK, AST, height, weight and academic record in their class ranking, returned to normal without taking any medication. They remained stable without relapse till this article was written.
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pmc-6245806-1
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A 42-year-old woman, who was admitted to the Department of Hematology, The Second Hospital of Hebei Medical University (Shijiazhuang, China) on May 6, 2016, presented with a one-month history of paleness and fatigue.
The patient was a farmer with a history of tuberculous pleurisy 24 years ago. The hematological analysis revealed the following: white blood cell count (WBC), 23.8 × 109/L; hemoglobin (Hb), 64 g/L; platelet count (PLT), 433 × 109/L. Bone marrow and peripheral blood smears identified the proliferation of lymphoblastic cells (87% of bone marrow nucleated cells). Karyotype analysis revealed a normal karyotype (46, XX) [20] (Fig. ). Immunophenotypic analysis by flow cytometry (FCM) revealed that blast cells accounted for 76.8%, which were positive for CD34, CD10, CD19, CD22 and HLA-DR, and negative for cIgM (Fig. ). Hence, the diagnosis of common B-cell acute lymphoblastic leukemia was confirmed.
The patient received a standard induction chemotherapy regimen with dexamethasone, vincristine, daunorubicin, cyclophosphamide and peg-L-asparaginase, and subsequently achieved partial remission. Merely 5% of lymphoblasts were observed in the bone marrow smear. After receiving the second induction chemotherapy with vincristine, cyclophosphamide, mitoxantrone, cytarabine and dexamethasone, the patient was discharged from the hospital (June 18, 2016).
On July 22, 2016, the patient was admitted to our hospital again for consolidation. The complete blood count revealed the following: WBC, 4.1 × 109/L; Hb, 82 g/L; PLT, 206 × 109/L. The bone marrow examination revealed 41% of lymphoblasts. Therefore, early relapse was diagnosed. After one cycle of CAM (CTX, Ara-c and 6-MP) and two cycles of MA (methotrexate and cytarabine), 26% of lymphoblasts was found in the bone marrow. The leukemia in the patient was relapsed and refractory. Therefore, CAR-T therapy was introduced to the patient.
After the patient provided consent, 100 mL of peripheral blood was collected to prepare the anti-CD19 CAR-T cells. The CAR-T cells were engineered by Hebei Senlangbio Technology Co., Ltd. (Shijiazhuang, China). The construct of CD19-CAR comprised of the following: anti-CD19 scFv (FMC63), the CD28 transmembrane domain, 4-1BB costimulatory, CD3zeta activation domains, T2A autocleavage sequences, and endodomain-truncated EGFR. Three lentivirus package systems were used (psPAX2, pMD2.G and pLenti-EF1-CAR19), and these were co-transfected with JetPRIME (Polyplus Transfection) in 293FT cells. The lymphodepleting chemotherapy with the FC regimen (30 mg/m2 of fludarabine, day − 4 to − 2; 30 mg/kg of cyclophosphamide, day − 3 to − 1) started on November 5, 2016 (day − 4). On day zero, the patient received an infusion of anti-CD19 CAR-T cells, which were transfected by the Senl-B19 lentiviral vector to express the anti-CD19 CARs, and expanded with IL-2 and IL-7. The total dose was 5.0 × 105 CAR-positive T-cells/Kg. Body temperature, C-reactive protein, CAR-T cell number in peripheral blood, the copy of CAR DNA, and leukemia cell level in the bone marrow were detected by FCM, as presented in Figs. and .
On the day of reinfusion of CAR-T cells, the patient developed fever with a temperature of 38.4 °C. The temperature was lowered by NSAIDs and returned to normal after a week. The fever regenerated on the 14th day after transfusion, and body temperature returned to normal after 3 days of piperacillin treatment. On December 2, 2016, the blood examination revealed the following: WBC, 3.2 × 109/L; Hb, 80 g/L; PLT, 354 × 109/L. On December 6, 2016 (day 28), no lymphoblast was found in the bone marrow smear. The minimal residual leukemia examined by FCM was also negative, since no CD19+CD34+ lymphocyte was found in the bone marrow.
The patient has a human leukocyte antigen (HLA)-full matched brother, who was willing to be a donor for the hematopoietic stem cell transplantation (HSCT). On December 11, 2016, the patient underwent allogeneic transplantation with granulocyte colony-stimulating factor mobilized bone marrow cells plus peripheral blood hematopoietic stem cells from the patient’s brother. The preconditioning regimen was myeloablative, including cytosine arabinoside (2 g/m2) on day − 9, busulfan (3.2 mg/kg/day) on day − 8, − 7 and − 6, cyclophosphamide (1.8 g/m2/day) on day − 5 and − 4, and semustine (250 mg/m2) on day − 3. The graft-versus-host disease (GVHD) prophylaxis was based on cyclosporine, mycophenolate mofetil (MMF), and a short course of methotrexate. Donor mononuclear cells of 8 × 108/Kg, including CD34 positive cells of 2.88 × 106/Kg, were transfused. Neutrophil and platelet engraftments were observed on day + 10, while a full-donor-chimerism of 97.5% was evidenced on day + 28.
On day + 28, the patient had a seizure, accompanied by thrombocytopenia. Then, red-cell fragments were found in the peripheral blood smear. Other important abnormal results included elevated levels of serum lactate dehydrogenase (LDH), positivity for proteinuria (240 mg/dL), and a negative Coombs test. All these led to the diagnosis of transplantation-associated thrombotic microangiopathy (TA-TMA). Cyclosporin A was discontinued, a high dose of methylprednisolone (10 mg/kg) was used and tapered, and therapeutic plasma exchange (TPE) was performed daily for the first week, and on every second day for the second week, followed by plasma infusion every 3 days for 2 weeks. On August 4, 2017, no sign of seizure was present, blood examination revealed a WBC of 9.9 × 109/L, a Hb of 77 g/L and a PLT 20 × 109/L. Furthermore, the bone marrow smear revealed no lymphoblasts, and the minimal residual disease (MRD) assayed by FCM was negative. The DNA chimerism revealed a full-donor type, and the patient was discharged from the hospital.
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pmc-6245809-1
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Herein, we describe an 81-year-old male with concomitant metastatic melanoma and pcALCL whose disease progressed on nivolumab and who then developed Kaposi’s varicelliform eruption following one cycle of T-VEC.
The patient had a complicated past medical history including coronary artery disease, treated with a coronary artery bypass graft, cerebral vascular accident following a left knee arthroplasty with residual partial aphasia, and a low-grade CD5+ B-cell lymphoproliferative disease, presenting as a large pleural effusion, which was put in a complete remission following 6 cycles of bendamustine and rituximab (R-Benda) (Figure ). Nearly two and a half years following completion of R-Benda, the patient developed ulcerative plaques on his chin, scalp, lip, right inner canthus and penile foreskin (Figure A-C). A skin biopsy was obtained and was consistent with an anaplastic lymphoma kinase (ALK)-negative, CD4+ CD30+, PD-1−, primary cutaneous anaplastic large cell lymphoma (ALCL). In addition to highlighting the cutaneous plaques of ALCL, a staging positron emission tomography-computed tomography (PET-CT) scan demonstrated a 2.7 × 1.5 cm fludeoxyglucose (FDG) avid right axillary lymph node. A core needle biopsy of the lymph node demonstrated melanoma. Five of 10 lymph nodes were positive for melanoma upon a right axillary lymphadenectomy.
A subsequent dermatological evaluation revealed a new 1 cm red/bluish nodule on the right forearm. Surgical excision of the lesion confirmed a 4.7 mm thick, nodular, BRAF wild-type melanoma. Concurrent with the diagnostic work up for his melanoma, the patient underwent targeted radiotherapy to the cutaneous ALCL lesions with excellent response. Following excisions of the right forearm and right axillary melanoma, a re-staging PET-CT demonstrated no additional areas concerning for metastatic melanoma.
The patient was then started on nivolumab for his resected, but high-risk, melanoma. At the time of nivolumab initiation, the patient had pink ulcerative, but improving, plaques of ALCL on his right tragus, chin, left preauricular skin and penile foreskin. He also had faint scaly plaques, without ulceration of the bilateral peri-ocular skin. On cycle 2 day 8 of nivolumab he developed new ulcerative plaques on his upper lip, left upper arm and left pre-auricular skin (Figure E) and worsening scale and erythema of his face that were consistent with progressing ALCL. Two new lesions consistent with melanoma recurrence were also noted on the right wrist at the site of the previous melanoma excision. Due to apparent worsening of his ALCL on nivolumab, the decision was made to treat concurrently with radiotherapy and brentuximab. Given the early time point, the appearance of new melanoma nodules were not considered to be a nivolumab failure, and he was continued on therapy.
On cycle 3 day 1 of nivolumab, the patient was given an initial dose of brentuximab vedotin. He also received external beam radiotherapy to his upper lip, left ear and left upper arm. On cycle 3 day 11, the patient was admitted for clostridium difficile-toxin positive colitis, which improved with oral vancomycin. A restaging PET-CT during cycle 3 demonstrated multiple new pulmonary nodules concerning for metastatic melanoma. On cycle 3 day 20, the patient was admitted again secondary to worsening fatigue and a concern for cellulitis surrounding a radiotherapy-treated ALCL lesion of his left upper arm. The patient was treated with antibiotics and improved. During this admission, new ulcerative plaques were noted on the scalp concerning for progression of the ALCL and cutaneous lesions of metastatic melanoma were identified on the right arm and right chest.
Due to worsening disease and declining performance status, the patient’s treatment options were limited. Ipilimumab was deemed inappropriate due to the recent clostridium difficile infection and worsening fatigue. There was also concern that his pcALCL was being exacerbated by ICB and that treatment with brentuximab may have accelerated the melanoma. Thus, after discussing the risks and benefits, the decision was made to discontinue brentuximab and treat the in-transit lesions of melanoma with talimogene laherparepvec concurrently with nivolumab. On cycle 4 day 1 of nivolumab, the patient was treated with 1.7 mL of 1 million plaque-forming-units/mL to three melanoma lesions on the right forearm and two on the right chest. At that visit, the patient was noted to have worsening erythema and scale of his upper extremities, upper chest and face. This was thought to be multifactorial, with asteatosis cutis a prominent feature, as well as either an ICB-related dermatitis or worsening pcALCL. On cycle 4 day 3, the patient presented to clinic febrile (temperature 103.0 F), fatigued and was noted to have a leukocytosis (21,200 white blood cells per microliter). He was subsequently hospitalized for 4 days and the presentation was attributed to an AE of T-VEC. During the admission, the patient had a skin biopsy of the worsening erythema and scale on his face (Figure F), which demonstrated features consistent with pcALCL.
In anticipation of a second cycle of T-VEC, the patient returned to clinic on cycle 4 day 22. On examination, a diffuse eruption of eroded papules was noted on his bilateral upper extremities, chest, flank and back (Figure A-C). A few intact vesicles were visible on the right forearm. The lesions were notably asymmetrical in distribution, with the highest density occurring on the right upper arm and right chest. The patient was afebrile and reported mild pruritus of the eruption, which he reported began a few days previously. A Tzanck smear was performed from one of the intact vesicles. Multinucleated giant cells with cytopathic changes were noted (Figure ). Due to concern for Kaposi’s varicelliform eruption (KVE), the patient was started on intravenous (IV) acyclovir. A direct fluorescence antibody test performed on a vesicle confirmed HSV1 infection. A skin biopsy demonstrated epidermal ulceration with acute inflammation and viral cytopathic effects. HSV I/II-specific immunoperoxidase stain was positive, while a specific immunostain for VZV was negative. HSV viremia was not detected by polymerase chain reaction. He was given 48 hours of IV acyclovir and when the vesicles had completely crusted over, he was discharged on a 14-day course of oral valacyclovir. He experienced a complete resolution of his KVE; however, due to his progressing melanoma, ALCL and declining performance status, the patient was transitioned to hospice care.
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pmc-6245818-1
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A 65-year-old man was referred to our hospital because of hyperproteinaemia, eosinophilia, anaemia, and proteinuria after a 2-week history of slight fever, fatigue, and malaise.
On admission, his mental status was normal, body temperature was 36.5 °C, pulse was 73 bpm and regular, and blood pressure was 118/75 mmHg. A physical examination revealed eruption and oedema in his lower extremities; however, no abnormal signs were observed in the lungs, heart, or abdomen. His lymph node and thyroid gland were not swollen. The laboratory findings on admission are summarized in Table . In brief, the eosinophil count was markedly increased (50%). The IgG and IgG4 levels were markedly increased (6380 and 2430 mg/dL, respectively). Urinalysis revealed massive proteinuria (3.5 g/day) with haematuria (5–10 per high-power field), and the β2-microglobulin level was 2863 ng/mL. Chest radiography revealed ground-glass opacities in the lower lung field. Chest computed tomography (CT) revealed bronchial wall thickening and ground-glass opacities in the right middle and lower lobes of the lung. Abdominal CT revealed bilateral renal enlargement.
A renal biopsy was performed. Light microscopy revealed 3 global scleroses and no crescent within the 9 glomeruli. In the interstitium, severe infiltration of plasma cells and eosinophils, with storiform fibrosis and infiltration of numerous IgG4-positive plasma cells (IgG4-/IgG-positive plasma cell ratio > 50%) were observed (Fig. a, b). In the functioning glomeruli, the GBM had a bubbling appearance with spikes but without significant mesangial cell or matrix proliferation (Fig. c). Direct fast scarlet staining was negative.
On immunofluorescence, the expression of IgG and complements was negative; however, IgA was positively expressed in a granular pattern along the GBM. An IgA subclass analysis revealed significant monoclonal deposition of IgA1-λ (Fig. ). We cut the frozen sections of renal biopsy specimens several times for other purposes such as immunostaining. Therefore, the last cut section was used for IgA subclass analysis, and there were few residual tissues and only an obliquely cut segment of the glomerulus remained. For this reason, we believe that it seemed that only a segment of the glomerulus stained with IgA1. Immunofluorescence staining for antibodies to M-type phospholipase A2 receptor (PLA2R) was negative (data not shown).
Electron microscopy revealed GBM thickening, widespread podocyte effacement, and irregular and small non-organized and non-Randall-type granular electron-dense deposits in the GBM (Fig. ) that were shaped like snow leopard spot-like pattern. On the basis of these findings, the diagnosis was IgA1-lambda-type non-Randall monoclonal immunoglobulin deposition disease associated with membranous features in a patient with IgG4-related TIN, according to the criteria for IgG4-RD []. Corticosteroid therapy was initiated with 3 days of 500 mg intravenous methyl-prednisolone, followed by 40 mg/day prednisolone. After the treatment, the oedema, eruption, and eosinophilia remarkably improved immediately. The serum creatinine level decreased to 1.0 mg/dL, and IgG and IgG4 levels decreased to within their normal ranges. However, massive proteinuria persisted.
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pmc-6245822-1
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The patient was a 20-year-old male who had been a low-birth-weight infant, and had a history of Klinefelter syndrome and pulmonary valve stenosis. He was introduced to our hospital for further examination of a liver tumor that was increasing in size. The tumor had been found incidentally after laboratory findings in a health checkup showed impairment of liver function. The patient had declined treatment due to his employment situation, and had instead been followed up for 1 year.
At the first visit, he was completely asymptomatic with normal vital signs. A physical examination revealed a palpable right upper mass without tenderness. No symptom related to Cushing syndrome was observed. In blood tests, hepatitis B virus surface antigen and hepatitis C virus antibody were negative. Liver function tests indicated mild dysfunction. Regarding tumor markers, serum alpha-fetoprotein (AFP) and carcinoembryonic antigen (CEA) were normal; however, neuron-specific enolase (NSE) was elevated.
Ultrasonography showed a large low-echoic solid tumor with a vertical diameter of > 80 mm with partial calcification implied by an acoustic shadow in an anterior lesion of the liver. A computed tomography (CT) scan of the chest, abdomen, and pelvis revealed an 81 × 76 × 72 mm large, heterogeneously enhanced mass in the right lobe of the liver with dense partial calcification (Fig. a). Subsequent positron emission tomography (PET)/CT showed a large hepatic mass in the right lobe with a maximum standardized uptake value (SUV) of 22.4 and no extrahepatic metastasis. In magnetic resonance imaging (MRI), most of the tumor was weakly enhanced in T1-weighted images and strongly enhanced in T2-weighted images. Part of the tumor had early enhancement and washout in enhanced MRI. These findings suggested HCC, and especially fibrolamellar HCC, but without evidence of distant metastasis.
Right hepatic lobectomy and cholecystectomy were performed 11 months after the initial detection of the tumor. The patient received no adjuvant chemotherapy or radiotherapy. The postoperative course was characterized by respiratory failure that required reintubation on postoperative day (POD) 2. X-ray and bronchofiberscopy showed pneumoniae due to pulmonary atelectasis and pulmonary edema. The subsequent hospital course was uneventful. On POD 7, a CT scan of the abdomen was interpreted as negative for hemoperitoneum and tumor recurrence, and the patient was discharged on POD 12.
The patient was followed up as an outpatient and received several examinations. On POD 62, a CT scan showed multiple, obscure, and circumscribed recurrent lesions in the remnant liver with contrast enhancement. The largest of these lesions had a diameter of 42 mm in segment 1 (S1) (Fig. b). In addition, a hypermetabolic para-aortic lymph node with possible metastasis was identified. On PODs 70 and 73, the patient underwent transcatheter arterial chemoembolization (TACE), but a second CT scan in the outpatient department on POD 84 revealed enlargement of recurrent tumors and the para-aortic lymph node. Chemotherapy (protocol for HCC) was started, but was unsuccessful because of side effects. At this time, there were no further surgical options and no other chemotherapy that was likely to be effective. Therefore, the patient received palliative care. The patient died 164 days after hepatectomy from tumor progression with development of progressive liver failure.
Grossly, the tumor was confined to the right liver lobe. The resected specimen weighed 1180 g. The lesion had a maximum diameter of 100 mm, and was a well-circumscribed solitary mass with multiple small calcifications that were sharply demarcated from surrounding uninvolved liver parenchyma (Fig. ). The surgical margin was tumor-free. Microscopically, the tumor was characterized by an organoid arrangement of cellular nests of epithelioid cells and areas of sheet-like cell overgrowth (Fig. a). These cells had oval-like nuclei with no clear nucleolus and eosinophilic cytoplasm. Transition zones between epithelioid and spindle cells were observed, and a framework of spindle cells surrounded nests of epithelioid cells (Fig. b, c). Bile ducts were not intermingled with the tumor region. There were extensive regions of necrosis and calcification (or ossification) in the center of the tumor (Fig. d).
In immunohistochemical staining, epithelioid cells were positive for CD56, cytokeratin AE1/AE3 (focal), WT-1 (diffuse or dot-like in cytoplasm), β-catenin (diffuse in nucleus), vimentin, NCAM, and NSE (Fig. a, b). Spindle cells in mesenchymal components such as the septum were diffusely stained with α-smooth muscle actin (α-SMA) (Fig. c). The AFP level was within the normal range. Staining for glypican-3 was negative. The proliferation index on MIB-1 (Ki-67) immunostaining was < 5%. Staining was negative for hepatocyte paraffin-1, CK7, adrenocorticotropic hormone (ACTH), estrogen receptor (ER), and progesterone receptor (PR). The morphological and immunohistochemical features led to diagnosis of CNSET.
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pmc-6245888-1
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A 29 year old woman with a previous history of migraine, mild asthma, congenital asymptomatic bicuspid aorta valve accidentally discovered during routine examination, missed abortion and anembryonic pregnancy was diagnosed with relapsing-remitting MS. She was treated with interferon beta1-b for five years, until it was decided to escalate the treatment due to new gadolinium enhancing MRI lesions and a sensory attack. The Expanded Disability Status Scale score was 2,0. Tests for tuberculosis, HIV, hepatitis B and C, routine blood and urine analyses, as well as respiratory examination and chest X-ray were negative. She had stopped smoking four years previously, and stopped using interferon beta 1b four months prior to the first alemtuzumab infusion because she wished to get pregnant.
The patient received standard premedication with 1000 mg methylprednisolone, 10 mg cetirizine, 1000 mg paracetamol and 400 mg acyclovir per day before each alemtuzumab infusion (12 mg per day). Prior to administration of alemtuzumab hypotension (70/35 mmHg) and bradycardia (45 beats per minute) was noticed and patient reported mild dizziness that improved after administration of Ringer’s acetate. A vasovagal reaction was suspected. For this reason, the alemtuzumab infusion was started at a low rate (12 ml/hour). Except mild headache that was treated with paracetamol and ibuprofen no infusion-associated reactions were observed the first day. Blood pressure and heart rate were normal during the alemtuzumab infusion.
At the end of the second alemtuzumab infusion, 24 h after the start of the first infusion, the patient developed chest pain on inspiration, shortness of breath, and cough. Four hours later she started coughing up bright red blood tinged sputum without clots. Body temperature, blood pressure, heart sounds and oxygen saturation were normal. Electrocardiogram showed sinus bradycardia at 48 beats/min. Auscultation revealed crepitations over the right lung, and chest x-ray showed corresponding shadowing. Platelet and leukocyte counts two hours after symptom onset were normal, and c-reactive protein was 26 mg/l (ref. < 5). Arterial blood gas analysis four hours after onset of haemoptysis was normal except for pO2 at the lower reference limit (11,0 kPa; ref. 11,0–14,4) and elevated lactate 1,3 mmol/l (ref.0,4-0,8). Haemoglobin fell from 12,0 g/dl (ref. 3, 7–15 g/dl) before the first infusion to 10,5 g/dl the day after onset of haemoptysis. Urinary analysis and serum creatinine remained normal.
Computed tomography (CT) pulmonary angiography performed shortly after onset of haemoptysis showed extensive bilateral upper and lower lobe opacities with centrilobular distribution and minimal (up to 5 mm) bilateral pleural effusions without evidence of pulmonary embolism (Fig. ). Interlobular septal thickening and dependent gradient were not present. The heart size was normal. At bronchoscopy performed 60 h after onset of haemoptysis the bronchoalveolar lavage (BAL) fluid was persistently macroscopically markedly bloody, without dilution on successive aliquots. There were no evidence of pathogenic bacteria, viruses, or atypical cells. Differential cell count of the BAL revealed 6% macrophages without hemosiderin inclusions on iron staining and 94% neutrophils indicating acute lung injury. Microscopy of BAL fluid showed many erythrocytes but counting in successive aliquots was not performed.
The patient remained stable from a respiratory point of view with normal vital parameters. No treatment for lung haemorrhage was given. Chest pain and haemoptysis resolved in two days. At discharge from hospital one week after onset of haemoptysis CT showed total resolution of pulmonary opacities and pleural effusions on the right side, and unchanged minimal amounts of pleural effusion on the left side. Her cough resolved after four weeks, and she has thereafter not experienced respiratory symptoms.
Alemtuzumab was discontinued after the second infusion. Her MS has remained clinically and radiologically stable, and new treatment has not been initiated because of prolonged lymphopenia (0,33 10*9/l 13 months after alemtuzumab infusions).
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pmc-6245891-1
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In mid September 2014, a 12-year-old boy presented at the Pediatric Emergency Department of the Fondazione IRCCS Policlinico San Matteo, Pavia, due to persistent fever, headache and diffuse pruriginous erythematous rash. The child’s mother reported that a few days before the onset of symptoms he went fishing near the family country-house in the province of Pavia (Po valley - Lombardy Region). On preliminary examination, his vital signs and general medical examination were normal. A mildly altered mental status was noted. Neurologic examination was unremarkable except for photophobia, without other signs of meningitis. Biochemical tests showed hemoglobin 12.8 mg/dl, lactate dehydrogenase 251 mU/ml (reference range: 125–220 mU/ml) and C-reactive protein 0.79 mg/dl (reference range: 0.00–0.50 mg/dl). Meningoencephalitis was suspected. The patient was thus hospitalized in the Pediatric Department and empiric antiviral and antibacterial therapies with acyclovir and ceftriaxone were promptly started. Cerebrospinal fluid (CSF) analysis showed 180 cells, glucose of 63 mg/dl (reference range: 40–70 mg/dl) and protein of 63 mg/dl (reference range: 20–45 mg/dl). Bacterial blood and CSF cultures were negative. CSF was tested by real-time RT-PCR and PCR for the following neurotropic viruses: Herpes simplex, Enterovirus, Polyomavirus JC, Herpesvirus 6, WNV, Phleboviruses and Flaviviruses. Furthermore, serum and urine were analyzed with WNV real-time RT-PCR and Flavivirus RT-PCR. The molecular investigation of neurotropic virus genome was negative in all the biological samples. Phleboviruses and WNV-IgM and IgG antibodies detection was performed in both serum and CSF samples. WNV-IgM tested positive both in serum and CSF while WNV-IgG were negative: this was confirmative for an acute WNV infection. An electroencephalogram (EEG), performed within 24-h after admission, revealed encephalitic-like bilateral slow waves and brain magnetic resonance imaging (MRI) did not reveal any abnormality.
The patient’s conditions remained stable and after seven days of hospitalization he was discharged with complete recovery and EEG normalization. At discharge, WNV-IgM and WNV neutralizing antibodies were positive and a WNV-IgG seroconversion was observed. The child was followed-up for 12 months, to monitor the clinical and neurological evolution and evaluate the WNV antibodies kinetics. A complete recovery with no neurological sequelae was observed. Serum WNV-IgM persistence was still evident 12 months after the onset of the disease.
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pmc-6245912-1
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A 41-year-old Japanese woman (gravida 2, para 0) had two previous miscarriages during the first trimester. She became pregnant via in vitro fertilization. Ultrasound findings during the second and third trimesters were not indicative of placenta accreta. She developed preeclampsia during the 36th week of gestation and underwent caesarean section. She delivered a healthy male infant (2178 g) with Apgar scores of 8 and 9 at 1 and 5 minutes, respectively. However, the placenta did not spontaneously separate; thus, the operator separated and gently removed the placenta from the uterine cavity manually. As adhesion was tight, placenta accreta was diagnosed. During the procedure, no uterine inversion or perforation was observed and there were no uterine cavity adhesions, such as those in Asherman’s syndrome. Manual removal was successfully performed.
Continuous bleeding was observed after removal of the placenta; thus, uterine gauze packing was performed, and the bleeding was stopped. On postoperative day 1, there was little bleeding; thus, the obstetrician removed the gauze. However, severe bleeding reoccurred. A balloon (Bakri® Balloon, Tokyo, Japan) was inserted into her uterine cavity and the bleeding stopped again. On postoperative day 3, the balloon was removed and there was no active bleeding this time.
One month postoperatively, she had no abnormal complaints. Two months postoperatively, her menses restarted. Four months postoperatively, we performed hysteroscopy. We detected an adhesion at the fundus of her uterus, in the location of the placenta accreta (Fig. ). Asherman’s syndrome was diagnosed.
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pmc-6245921-1
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In February 2016, a 57-year-old woman was admitted to our hospital for evaluation of a breast mass and multiple pulmonary nodules. AF18-fluorodeoxyglucose (FDG) positron emission tomography computed tomography (PET-CT) scan performed at the Shengjing Hospital of China Medical University showed a left breast mass with a FDG maximal standardized uptake value (SUVmax) of 4.23 (Fig. ), a left lower lung lobe (LLL) nodule measuring about 1.1 cm in diameter with increased FDG uptake (SUVmax = 2.79; Fig. ), and a right lower lung lobe (RLL) nodule measuring about 0.8 cm with normal FDG uptake (Fig. ). The LLL lesion was considered malignant, whereas the RLL lesion was not diagnosed as benign or malignant. Sequential surgery for resection of the breast cancer and LLL lesion was considered a reasonable course of action.
A left radical mastectomy was performed on March 2nd, 2016. Postoperative pathology showed ductal carcinoma in situ (high grade). Immunohistochemical (IHC) staining indicated that the lesion was estrogen receptor(ER) negative (Fig. ), progesterone receptor(PR) negative (Fig. ), C-erbB-2 positive carcinoma in situ (3 +; Fig. ) and thyroid transcription factor-1(TTF-1) negative(Fig. ). The margins were negative. Sentinel lymph node analysis revealed reactive hyperplasia in the axillary lymph node (0/5,0/10). The pathological stage was pTisN0M0, 0 stage according to AJCC version 7.0 []. EGFR gene analysis (Fig. ) revealed no mutations.
A left lower lobectomy with lymph node dissection was performed at our hospital in April 2016. Postoperative pathology identified a highly to moderately differentiated adenocarcinoma (gland bubble type, 90%; lepidic growth pattern, 10%). Cancer cells were not detected in the lymph nodes. By IHC analysis, the lesion was CK7 positive (Fig. ), P63 negative, napsin A positive (Fig. ), TTF-1 positive (Fig. ), ALK D5F3 negative, ALK negative, and Ki-67 positive (5%). EGFR gene analysis was performed in June 2016 and showed an L858R mutation in exon 21 (Fig. ). The pathological results of breast cancer (pTisN0M0,stage 0) and lung cancer (pT1aN0M0, stage IA according to AJCC version 7.0 []) showed that the patient has MPMT.
A chest CT scan performed 2 months after the left lower lobectomy showed that the small ground glass nodule in the RLL had increased in size compared with the scan performed 5 months earlier. Since the LLL lesion expressed mutated EGFR, the patient was prescribed gefitinib 250 mg daily starting in August 2016. A follow-up chest CT scan performed 40 days later showed a stable RLL nodule.
A right lower lobectomy with lymph node dissection was performed in our hospital in September 2016. Postoperative pathology identified an adenocarcinoma (alveolar type, 40%; growing along the alveolar wall, 60%). There was no lymph node metastasis and the surgical margins were clear. By IHC analysis, the lesion was CK A1 positive (Fig. ), CK5/6 negative, CK7 positive (Fig. ), P63 negative, P40 negative, napsin A positive (Fig. ), TTF-1 positive (Fig. ), CD56 negative, synaptophysin negative, and Ki-67 positive (10%). Analysis of the EGFR gene showed a deletion in exon 19 (Fig. ). The pathological TNM stage was pT1aN0M0, stage IA according to AJCC version 7.0 [].
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pmc-6246101-1
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A 6-month-old female infant (6000 g; 3rd percentile) with an unremarkable medical history was transferred to our pediatric ward because of postprandial vomiting for 5 days. On clinical examination, she was subfebrile (38°C) and had a distended but soft abdomen. No mass was palpable. Abdominal ultrasound suggested the possibility of intussusception. As an enema to reduce intussusception was no therapeutic option at our center, the abdomen was surgically explored. On laparotomy, blood-stained but clear ascites was found as well as intestinal adhesions. Moreover, we saw diffuse granulations, mesenteric lymphadenopathy, and ischemia of the terminal ileum (
and
). Peritoneal cultures were harvested, appendectomy was performed, and the intestinal lymph nodes were biopsied. Postoperatively, tuberculin intradermal reaction (IDRt) was positive at 9 mm and cultures grew tuberculous contagium. The Anti-TB treatment administered was: rifampicin, isoniazid, pyrazinamide, ethambutol (RHZE) for 2 months, then RH the following 4 months. This regimen was applied before receiving the biopsy results which showed tuberculoid follicular lesions (
and
). The patient was declared cured after this treatment. She had no abdominal symptoms after 2 years of follow-up.
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pmc-6246101-2
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An 8-month-old (7000 g; 3rd percentile) boy from Mauritania with no particular pathological history was transferred from a health center near the Senegal–Mauritanian border for the management of peritonitis. The clinical picture had evolved for 15 days, with the appearance of abdominal bloating associated with vomiting and cessation of bowel movements. On clinical examination, he had a temperature of 38°C, and was tachycardic at 148 beats per minute. He showed nutritional edema and flank dullness. His abdomen was distended and had peritonitis on palpation. Therefore, the boy was taken to the operating room. Surgical exploration revealed clear ascites associated with small intestinal granulations and adhesions. We performed adhesiolysis, biopsies, and drainage of the peritoneal cavity. Postoperatively, GeneXpert tests were performed on the peritoneal fluid and the IDRt was negative. Anatomopathological examination of the operative specimens revealed the presence of caseous necrosis compatible with peritoneal TB. The medical treatment and the evolution were identical to Case 1.
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pmc-6246436-1
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A 34-day-old female infant, weighing 2.3 kg, was admitted to our institute with severe respiratory distress. Physical examination demonstrated severe tachypnea (respiratory rate = 35-40/min), sweating during breastfeeding, peripheral cyanosis, and indrawing of the chest cavity with inspiration. Chest X-ray revealed only cardiomegaly (). Transthoracic echocardiography showed massive pericardial effusion with a space-occupying mass lesion, 5 × 4 cm in size, in the pericardial cavity on the right side. Based on the infant’s clinical condition and the echocardiographic findings, she was scheduled for emergency tumor removal.
The preoperative heart rate was between 130 and 140 bpm. The peripheral capillary oxygen saturation (SpO2) before induction was 88% and after the administration of 100% oxygen with a mask, SpO2 rose to 94%. Considering the infant’s age, severe respiratory distress, and cardiovascular instability, we performed awake-sedate intubation after the administration of 5 μg of fentanyl and topicalization of the tongue, larynx, and vocal cords with 15 mg of 1.0% lidocaine and a 3.0-mm Portex tracheal tube. Subsequently, the general anesthesia was maintained with a mixture of oxygen/air, isoflurane, 1-mg increments of atracurium, and 5 μg of fentanyl intermittently. Thereafter, an arterial line and a central venous line were established. Other monitoring modalities such as the bispectral index (BIS), capnograph, and pulse oximetry were utilized as well. Extracorporeal circulation was on standby. Via a median sternotomy with the preservation of the thymus gland, the pericardium was opened and a large quantity of cloudy yellow fluid was aspirated. A multicystic lesion, around 50 × 45 × 30 mm in size, was revealed. It covered most of the right ventricle and pressed on the right atrium ( and ). Furthermore, the mass was polycystic and attached to the right anterior wall of the ascending aorta. The whole tumor was successfully removed. The patient was extubated in the intensive care unit on the first postoperative day and was discharged from the hospital on the fifth postoperative day.
Histologically, it was a mature teratoma with the representation of all 3 germinal layers: endoderm 50%, mesoderm 25%, and ectoderm 25%.
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pmc-6246437-1
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A 67-year-old male patient, who had anterior myocardial infarction and undergone percutaneous coronary intervention on his left anterior descending artery in 2009, was referred to our cardiology clinic after the failure of the first cardiac resynchronization therapy defibrillator (CRT-D) implantation. He had also undergone percutaneous coronary intervention on his right coronary artery 2 years previously. He was diagnosed with a reduced ejection fraction (22%) heart failure after transthoracic echocardiography. His medications included enalapril (10 mg twice daily), carvedilol (25 mg), spironolactone (50 mg), acetylsalicylic acid (100 mg), and ivabradine (7.5 mg). Despite optimal therapy for 8 months, his resting dyspnea and pretibial edema worsened gradually and his exercise capacity was very limited (New York Heart Association functional class III). The 6-minute walking test was performed, and the result was reported to be 180 m. His electrocardiogram (ECG) showed sinus rhythm and left bundle branch block with a QRS of 152 ms (). The previous cardiology center attempted to implant a CRT-D in order to palliate the symptoms. Following the failure of the implantation, the patient was referred to our department for reassessment. Another implantation procedure was planned because the patient’s cardiac status sufficiently fulfilled the criteria for CRT-D implantation.
After the cannulation of the coronary sinus with an electrophysiology catheter, a coronary sinus access catheter was placed in the coronary sinus. The angiography of the coronary sinus was performed with a balloon catheter. Efforts were made to place the left ventricular lead in the coronary sinus; however, high tortuosity thwarted the attempts. During maneuvers to detect the middle cardiac vein, the posterior vein was observed to be draining directly into the right atrium (, , and ). The posterior cardiac vein was demonstrated to have a separate orifice by the retrograde filling of the coronary sinus via contrast injection into the posterior cardiac vein. A multipolar left ventricular lead was implanted in the posterior vein through the separate ostium from the right atrium (). After the implantation, no complication was detected in the chest X-ray. The QRS in the ECG after the procedure narrowed significantly (). Verification of the separate ostium was also provided via noninvasive venography using multi-slice computed tomography ().
The patient was stable and he was discharged under the same medical therapy on the fifth postoperative day. At 3 months’ follow-up, he was asymptomatic (New York Heart Association functional class I). The 6-minute walking test was repeated, and the result was reported to be 410 m. Accordingly, to the best of our knowledge, we are the first in the relevant literature to achieve a successful intervention by implanting a multipolar left ventricular lead in a separately originated posterior cardiac vein.
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pmc-6246438-1
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A 38-year-old left-hand dominant man woke up with acute pain and swelling in his left upper limb. He was a computer programmer and, additionally, used to work as a disc jockey in a local nightclub every weekend. He had no intrinsic risk factors for thromboembolic disease but spent prolonged periods of the day with his upper limbs in a relatively stationary position whilst using a computer keyboard.
Physical examination revealed erythema, heat, swelling, and tenderness localized to the ulna border of his left arm (). There was no systemic feature of illness.
Laboratory tests including routine full blood count, renal and liver profiles, inflammatory markers, thrombophilia test, and viral screen were unremarkable, with the exception of elevated D-dimer.
Venous duplex ultrasound of his left arm demonstrated compressible radial, ulnar, and brachial veins with decreased compressibility in the left axillary vein and confirmed the presence of thrombosis extending throughout the length of the vessel. Although there was no obvious symptom or sign of pulmonary embolism, we decided to proceed with computed tomography pulmonary angiography (CTPA), given the patient’s Wells score of 3 (proven DVT), which corresponds to an intermediate risk category for pulmonary embolism (score ≥2 and ≤6). CTPA showed no evidence of pulmonary embolism.
We treated the patient with full anticoagulation using subcutaneous low molecular weight heparin and oral warfarin, and his signs and symptoms gradually resolved over the next few days. On subsequent follow-up at 3 months, he remained well and asymptomatic and repeated venous Doppler ultrasound revealed complete resolution of the left axillary thrombosis. Following the guidelines from the American College of Chest Physicians, which recommend anticoagulation for a minimum of 3 months for all patients identified with uncomplicated primary upper extremity DVT, we discontinued his warfarin.
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pmc-6246439-1
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An 8-year-old girl, who suffered from chronic cyanosis, was referred to our hospital for further evaluation. The patient was well developed with normal growth. On physical examination, a 2/6 systolic heart murmur on the middle left sternal border and mild cyanosis (O2 saturation=80% by pulse oximetry) were the only findings. Her blood pressure and heart rate were 110/70 mmHg and 100 bpm, respectively. She had no history of stroke, dyspnea, dizziness, and syncope. Electrocardiography was normal, and there was no finding in favor of the enlargement of any chamber. Chest X-ray showed mild cardiomegaly with normal pulmonary vascular markings. Her hemoglobin and hematocrit levels were 14 g/dL and 41%, respectively. Transthoracic 2D echocardiography, via the segmental approach, demonstrated the normal continuation of the inferior vena cava to the right atrium. However, the orifice of the RSVC to the right atrium could not be visualized. Instead, it seemed that the RSVC was draining into the left atrium. The left atrium and the left ventricle were mildly enlarged (). The function of both ventricles was normal. Other echocardiographic findings were mild mitral regurgitation and trivial tricuspid regurgitation. The peak pressure gradient of the tricuspid regurgitation was 20 mm Hg, and there was mild pulmonary valve regurgitation. In addition, there were no intracardiac defects or shunts. The pulmonary veins were connected to the left atrium normally. The diagnosis was confirmed through contrast study with agitated normal saline. Injection via the left brachial vein showed the drainage of the SVC into the left atrium, no atrial septal defect, and no left SVC ( & ). Cardiac catheterization and angiography confirmed a systemic oxygen saturation rate of 80% and a normal pulmonary artery pressure (mean pulmonary artery pressure=15 mmHg). Because transthoracic echocardiography and catheterization confirmed the diagnosis (), there was no need to utilize any other imaging modalities.
Surgery was performed through median sternotomy. After aorto-bicaval cannulation and the initiation of cardiopulmonary bypass, cardiac arrest was induced by blood cardioplegia. The SVC was cut from the left atrium, translocated, and anastomosed to the right atrium, which was then augmented with an autologous pericardial patch. The patient was successfully weaned from cardiopulmonary bypass and had an uneventful recovery course.
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pmc-6246743-1
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The study involves two patients. The first is a 57-year-old Italian man who accidentally discovered to be affected, being asymptomatic. The single lesion at the right cerebellar hemisphere was diagnosed by an MRI exam performed after an accident (Figure ). No evidence of suspected affected relatives was reported by the patient and, therefore, he was classified as sporadic.
The second patient is an 84 year-old woman affected by hereditary CCM (Figure ; I-2) diagnosed about 20 years ago following recurrent epileptic seizures. No MRI scan images are available; however, from medical reports read, the presence of four cavernous lesions emerged and, particularly, two at the pontine region and two at the right parietal lobe. The woman could not provide anamnesis information on her parents. The study was also extended to other members of the family and, particularly, to three of her sons and five grandchildren. Three of her sons, as reported in Figure , are affected. The first born is a 60-year-old man (Figure ; II-1) who suffers from global transient amnesia without neurological deficits. MRI highlighted at least seven lesions, distributed among brainstem and both cerebellar hemispheres (Figure ). Diagnosis in one of the two second-born (a twin) Figure ; II-3) was performed subsequently to familial history. Despite MRI showing the presence of four CCM lesions at brainstem (Figure ) the 58-year-old patient is, to date, asymptomatic. No information is available about his twin. The younger daughter (Figure ; II-4) is 48 years old and manifests recurrent headaches, right hemiparesis, and hemi-hyperesthesia. These symptoms are due to five CCM lesions affecting brainstem and both cerebral hemispheres (Figure ). Regarding the five grandchildren, MRI was positive only for one of them (Figure ; III-13) who, however, is asymptomatic. In detail, she carries three lesions, two in the area of the posterior limb of the right internal capsule and one in the left temporal lobe (Figure ). The patients involved in the study were fully informed and informed consent was obtained in accordance with the Declaration of Helsinki. Written informed consent for the publication of this study was obtained from the participants. The study was approved by our local Ethical Committee A.O.U. G. Martino Messina.
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pmc-6247407-1
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A 48-year-old bisexual gentleman presented to the eye clinic with a one week history of redness, discharge and reduced vision in his left eye. He had no past ocular history, and no recent systemic upset. He had a background of schizophrenia and obsessive compulsive disorder for which he received zuclopenthixol intramuscular injections every three weeks. Recent sexual history revealed multiple sexual partners, both male and female, without use of protection.
On examination visual acuity unaided was 0.20 LogMAR right eye (improving with pinhole suggesting untreated refractive error) and Hand Movements (HM) in the left eye, no improvement with pinhole. Anterior segment examination revealed profuse mucopurulent discharge from the left eye, with upper and lower eyelid swelling and conjunctival injection. The cornea was clear. The right anterior segment was normal. Sterile swabs were used to obtain conjunctival surface samples from the left eye for bacterial culture (Sterilin Charcoal Transport Swab, Thermo Fisher Scientific, Loughborough, UK), for Herpes Simplex Virus PCR testing (Remel, Lenexa, USA) and Chlamydia trachomatis testing (COBAS PCR Dual Media Swab, Roche Diagnostics Limited, West Sussex, UK). The patient was commenced on 2 hourly Ofloxacin 0.3% eye drops and chloramphenicol 1% ointment 4 times daily and discharged home.
The patient returned two days later with worsening symptoms that were now present bilaterally. He had not used any of the prescribed eyedrops. Examination of the anterior segment showed bilateral profuse mucopurulent discharge, lid swelling and conjunctival injection. The right cornea was clear but the left cornea now showed two areas of thinning peripherally in the superior and temporal regions ( and ). Due to compliance issues with initial treatment and worsening of the clinical signs, the patient was admitted to the ward for further investigations and management.
Initial test results were positive for Chlamydia trachomatis and the clinical suspicion of co-infection with Neisseria gonorrhoeae led to further samples being taken. These were conjunctival surface swab samples from the everted upper lid and lower lid, that were directly placed on to sterile glass slides for Gram stain, followed by inoculation of chocolate agar and blood agar plates. An additional conjunctival swab sample (Transwab, Medical Wire, Wiltshire, UK) was sent for polymerase chain reaction (PCR) for Neiserria gonorrhoeae testing (COBAS PCR Dual Media Swab Kit, Roche Diagnostics Limited, West Sussex, UK). The gram stain was performed according to standard procedures and showed gram-negative diplococci consistent with a diagnosis of N. gonorrhoeae infection (), later confirmed by a positive PCR test result.
Following a discussion with the microbiologist the patient was commenced on intravenous ceftriaxone 2mg once daily, oral azithromycin 500mg once daily for five days and topical levofloxacin preservative-free drops to both eyes hourly.
48 hours after commencing treatment there were signs of clinical improvement. Visual acuity had risen to logMAR 0.50 in the left eye, unaided. Corneal thinning had stabilized and re-epithelialization of the thinned cornea was observed. The patient was referred to the Genito-urinary Medicine team for systemic test of cure and contact tracing.
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pmc-6247437-1
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A 55-year-old Thai man presented with dyspnea for 1 day. The patient had a history of odynophagia and dysphagia for a week. He had a history of poor oral hygiene. On physical examination, he had low-grade fever (38°C), dyspnea, and marked swelling of the base of tongue (BOT) with partial occlusion of the oropharyngeal airway. Complete blood count (CBC) showed his white blood cell (WBC) count was 9300/mm3 with predominant neutrophil. Orobuccal computed tomography (CT) scan revealed the large abscess at the BOT. Tracheostomy and surgical drainage were performed. Streptococcus viridians was identified from pus culture. Amoxicillin-clavulanic acid 1.2 g 8 hourly plus ceftriaxone 2 g once daily were administrated for 2 weeks with an excellent outcome. The patient could be decannulated after the sixth day of treatment with airway patency.
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pmc-6247437-2
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A 52-year-old Thai man presented with a painful tongue and odynophagia for 7 days. The patient had a history of well-controlled diabetes mellitus (DM). He denied either trauma or infection at orobuccolingual regions. Also, the patient had poor oral hygiene. Physical examination showed the patient was afebrile (36.6°C). The antero-lateral aspect of the tongue was swollen and fluctuation. The airway was patent. CBC showed WBC was 4500/mm3 with neutrophilic predominance. Surgical drainage was performed under local anaesthesia. The pus culture did not show any organisms. Empirical antibiotics with amoxicillin-clavulanic acid 1.2 g 8 hourly and ceftriaxone 2 g once daily were administrated for 10 days, and the outcome was good.
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pmc-6247437-3
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A 52-year-old Thai woman reported having odynophagia and dysphagia for 1 week. She took amoxicillin for 5 days without signs of improvement. Her symptoms worsened, as did limitation of her tongue movement. She denied local trauma of orobuccolingual regions. Examination showed that her floor of mouth (FOM) and BOT were swollen without airway obstruction. She was afebrile (36.6°C), but her WBC was 14500/mm3. Contrast-enhanced CT scan demonstrated an abscess at the sublingual space and BOT. The pus was drained with the patient under general anaesthesia. The organism was identified as Acinetobacter lwoffii. Clindamycin 600 mg 8 hourly and ceftriaxone 2 g once daily were prescribed for 2 weeks, with good clinical response.
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pmc-6247437-4
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A 46-year-old Thai woman with poorly controlled DM presented to the emergency department with dyspnea for 1 day. She had odynophagia and dysphagia for 4 days. She denied a history of trauma at orobuccolingual regions. On physical examination, her body temperature was 37.5°C. Limitation of tongue movement and swelling of the tongue and FOM were observed. The oropharyngeal airway was partially obstructed. Contrast-enhanced CT scan showed the abscess confined to the ventral aspect of the tongue with sublingual space cellulitis (). CBC showed WBC was 12,100/mm3 with neutrophilic predominance. The patient underwent tracheostomy and surgical drainage under general anaesthesia. The tracheostomy tube was safety removed 5 days after the operation. Beta-haemolytic non-group A, B, D Streptococcus Spp. was identified; then, clindamycin 600 mg 8 hourly and ceftriaxone 2 g once daily were administrated intravenously, and all symptoms were completely resolved 2 weeks after initiation of treatment.
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pmc-6247437-5
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A 58-year-old Thai male was admitted due to pain in the BOT with referred pain to the ear for 1 week. He had poor oral hygiene. He took amoxicillin for 5 days without clinical improvement. On physical examination, he was afebrile (37.3°C). Pain was detected at the left side of BOT just behind the circumvallate papillae with marked fluctuation. The other orobuccal regions were normal without evidence of airway obstruction. CBC showed WBC was 5500/mm3. Contrast-enhanced CT scan demonstrated an abscess at the left posterior tongue (). In addition, an incidental thyroglossal duct cyst was identified without sign of infection (). The patient underwent surgical drainage under general anaesthesia. Streptococcus viridans was identified. Amoxicillin-clavulanic acid 1.2 g 8 hourly and ceftriaxone 2 g once daily were prescribed for 2 weeks, with an excellent outcome.
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pmc-6247437-6
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A 59-year-old Thai woman reported having mass within her tongue for 10 days. She had mild degree of pain and took amoxicillin-clavulanic acid for 7 days. Her tongue's mass was still the same size while the pain was resolved. Her oral hygiene was good with no active dental and periodontal conditions. Physical examination showed the patient was afebrile (37°C). The firm mass within the antero-midline of the tongue of about 1.5∗1.5 cms in size was palpated without any sign of inflammation. CBC showed WBC was 5800/mm3 with neutrophilic predominance. The patient was informed about the treatment option and she decided to remove the mass under general anaesthesia. Intraoperative finding showed well circumscribed loculated abscess and the pus was drainage. Pathologic report for surrounding tissue was acute and chronic inflammation with abscess formation. The pus culture did not show any organisms. Empirical antibiotic with amoxicillin-clavulanic acid 1.2 g 8 hourly was administrated for 1 week, and the outcome was good.
All patients' demographic data are described in , and the details of investigation, treatment and clinical outcome are shown in .
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pmc-6247484-1
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A 64-year-old Hispanic male with diagnosis of pulmonary coccidioidomycosis 2 years prior at another institution and placed on therapy with 400 mg fluconazole daily for 1½ years. Initial serum coccidioidal immunodiffusion of IgM (immunoglobulin) and IgG were weakly reactive with complement fixation titers of 1:4. Symptoms resolved, and his physician decreased fluconazole to 200 mg daily for 4 months. He did well for 1 month until he developed left-sided headaches. After 2 weeks, he had 2 episodes of left arm and leg weakness without ability to walk and lower right facial palsy over a period of 10 minutes.
In the emergency department, while having his vitals taken, the patient had another episode of lower right facial palsy and left-sided weakness that resolved in 5 minutes. Computed tomography scan of brain without contrast as well as computed tomography angiogram of head and neck were completed and were unremarkable. Three hours later, the patient had another episode of right facial droop and left-sided weakness, followed by new-onset slurring of speech, resolving in 5 minutes. Magnetic resonance imaging of the brain showed no infarcts or intracranial hemorrhage, but it did show increased peripontine enhancement with several nodular enhancements in the basilar area suspicious for coccidioma (). Lumbar puncture demonstrated opening pressure of 140 mm H2O, white blood cells 240 (34% lymphocytes, 39% monocytes, 18% neutrophils, 4% eosinophils, and 5% basophils), elevated protein 127 mg/dL (normal = 14-45 mg/dL), glucose 38 mg/dL (normal = 40-75 mg/dL), and coccidioidal compliment fixation titer of 1:4 diagnostic of coccidioidal meningitis. Serum coccidioidal immunodiffusion IgM and IgG were reactive with a compliment fixation titer of 1:16 (). He had a total of 4 cerebrovascular TIAs that were increasing in intensity and symptomology. He was placed on fluconazole 1000 mg daily and a dexamethasone 20 mg daily for 7 days then tapered by 4 mg every 4 days. He was discharged to be followed in clinic.
He had been and continues to be compliant with fluconazole therapy. At 2-month hospital visit for skin and soft tissue infection of left ankle unrelated to coccidioidomycosis, serum coccidioidal fixation titers were improved to 1:4. At 4-month follow-up, the patient had been asymptomatic. Lumbar puncture in office demonstrated white blood cell count of 50 (81% lymphocytes, 16% monocytes, 1% neutrophils, 1% eosinophils, and 1% basophils), protein 66 mg/dL (normal = 15-45 mg/dL), glucose 43 (normal = 40-75 mg/dL), and coccidioidal fixation titer of less than 1:1. However, serum coccidioidal fixation titers were 1:8 ().
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pmc-6247568-1
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A 57-year-old female visited a respiratory internal physician due to suspected lung cancer (based on a mass screening chest X-ray examination). She did not have any symptoms. The chest X-ray showed a tumor shadow in the upper-middle field of the right lung with pleural effusion and a tumor shadow in the upper field of the left lung (). Computed tomography (CT) of the neck and chest revealed that the tumor shadows had been caused by a substernal goiter connected to the thyroid gland in the neck. According to the patient, she had been diagnosed with a goiter about 23 years ago, and it was followed up, but the follow-up process had been discontinued several times. After about 20 years, she visited our hospital for surgical treatment.
In a physical examination, the palpable thyroid gland was found to be diffusely swollen and soft and exhibited poor mobility. The lower pole of the thyroid was not palpable.
A blood examination revealed normal thyroid function, a thyroglobulin level of 352 ng/ml, and negativity for the thyroglobulin antibody.
Ultrasound showed that the cervical thyroid gland was diffusely enlarged and exhibited multiple regions of cystic degeneration, but no obvious malignant findings were observed.
CT of the neck and chest () showed the diffusely swollen thyroid gland and a substernal goiter, which extended to both sides of the thorax. Specifically, it extended to the bifurcation of the trachea on the dorsal side of the superior vena cava, the innominate vein, the aortic arch, and the ventral side of the trachea. The width of the goiter at the mediastinum was 145 mm (length: 80 mm, thickness: 80 mm). The right side of the substernal goiter was bigger than its left side. The interior of the lesion was heterogeneous, and calcification was seen in part of it. The goiter had compressed the trachea in the mediastinum, and the lumen of the trachea measured 6 mm in diameter at its narrowest point. Pleural effusion was noted in the right thorax. We performed 18F-fluorodeoxy glucose positron emission tomography to determine the malignancy of the substernal goiter, but no radiotracer accumulation was observed.
We also conducted a pathological examination. Fine-needle aspiration cytology of the cervical thyroid gland resulted in the lesion being classified as of “indeterminate significance,” and a pathological examination of a needle biopsy sample from the same site led to the lesion being diagnosed as a follicular neoplasm. Fine-needle aspiration cytology of the right pleural effusion demonstrated that it was benign.
The patient underwent total thyroidectomy using a transcervical and full sternotomy approach. The anesthesiologist intubated the patient with a bronchoscope. Although tracheal stenosis was observed, intubation was performed smoothly. Later, the tracheal tube was replaced with an NIM™ EMG endotracheal tube so that intraoperative nerve monitoring could be performed. The patient was placed in a supine position with her neck well extended. A cervical skin incision was made, and a median chest midline incision and full sternotomy were performed. First, we identified the bilateral vagal nerves and confirmed the absence of paralysis with the NIM™. As a preparation for the resection of the substernal goiter, the major blood vessels, including the innominate vein, brachiocephalic trunk, superior vena cava, and left subclavian artery, were carefully separated from the substernal goiter, and then thyroidectomy was performed ().
The right superior thyroid pedicle and right middle thyroid vein were ligated and dissected to allow the right thyroid lobe to be rotated to gain a view of the recurrent laryngeal nerve (RLN) from the lateral aspect of the thyroid gland, but the goiter prevented the right thyroid lobe from being rotated. It was difficult to identify the right RLN, so we decided to try to exteriorize the left thyroid lobe, which was smaller than the right thyroid lobe. The left superior thyroid pedicle and the left middle thyroid vein were ligated and dissected. The left thyroid lobe was more mobile than the right thyroid lobe, and the left RLN could be identified by rotating the left thyroid lobe in the medial direction. The NIM™ was effective at identifying the RLN. After identifying the left RLN, the left lower thyroid artery was ligated and dissected. The left RLN was carefully separated from the dorsal side of the left thyroid lobe and the substernal goiter so as not to cause any damage. The substernal goiter, which was connected to the left thyroid lobe, was pulled in the cranial direction, and the part adhering to the surrounding tissue, particularly the tissue between the goiter and the innominate vein, was dissected by ligation and coagulation with an energy device. Subsequently, the left thyroid lobe was also separated from the trachea. The exteriorization of the left thyroid lobe improved the mobility of the right thyroid lobe, and the right RLN was identified by dislocating the right upper pole to the caudal side. We carefully separated the right RLN from the goiter and ligated and dissected the right lower thyroid artery. We pulled the substernal portion of the right thyroid lobe gradually; separated the tissue connected to the goiter, including the left thyroid lobe; and succeeded in moving the substernal goiter in the cranial direction. The remaining attachments between the right thyroid lobe and trachea were broken, and a total thyroidectomy was conducted. We found three parathyroid glands had adhered to the resected thyroid gland, so we performed autotransplantation using the sternocleidomastoid muscle. The wound closed after drains were inserted in the neck and mediastinum. After the surgery, the patient was extubated immediately because no respiratory tract problems (e.g., tracheomalacia) were noted. The total duration of the operation was 9 h and 22 min, and the total amount of intraoperative blood loss was 3298 ml. The resected thyroid weighed 614 g ().
Postoperative transient hypoparathyroidism was observed. Routine treatment with calcium (3 g daily orally) and 1 alpha-hydroxyvitamin D3 (2 μg daily orally) was administered. The patient was discharged home on the 9th postoperative day on levothyroxine (100 μg daily orally). A histopathological examination did not reveal any signs of malignancy, and so the lesion was diagnosed as an adenomatous goiter.
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pmc-6247618-1
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An 11-year-old Caucasian girl presented to the Pediatric Hospital in Damascus with a 2-month history of diffuse abdominal pain, yellowish vomiting after meals, a low-grade fever (38.5°) that responded to anti-pyretic medications, malaise, and polyuria. A physical examination on admission revealed mild pallor, light pigmentation on the lips, and a body mass index (BMI) of 17.9 kg/m2. Her vital signs were as following: blood pressure 80/50 mmHg, temperature 37.0°, heart rate (HR) 100/minute, and respiratory rate (RR) 20/minute. There was no jugular venous distention, no lymphadenopathies and no organomegalies. Heart and lung auscultation were normal. She had no signs of peripheral edema. Her Mini Mental State Examination score was 26/30 and her Glasgow Coma Scale was 15/15. Her muscle strength, tone, and reflexes were all normal. Sensory examination and cranial nerves were normal. She had been treated with nitrofurantoin for recurrent urinary tract infections. Her medical history included no other medications. There was no significant family, social, or environmental history. Her Caucasian parents were not related.
A complete blood count (CBC) showed reduced white blood cells (WBC) of 3940 cells/mm3 with neutrophils/lymphocytes (N/L) of 52/26, hemoglobin (HB) 9 g/dL, platelets (PLT) 240,000/mm3, and mean corpuscular volume (MCV) 77 fL. Laboratory studies showed: sodium 129 mEq/L, potassium 4.53 mEq/L, creatinine 0.97 mg/dL, chloride 105 mEq/L, ionized calcium 1.25 mmol/L, alkaline phosphatase (ALP) 223 IU/L, fasting glucose 97 mg/dL, glycated hemoglobin (HBA1c) 4%, C-reactive protein (CRP) 19.6 mg/L, and erythrocyte sedimentation rate (ESR) 112 mm/hour. Thyroid-stimulating hormone (TSH) and free thyroxine (FT4) were within normal range. Serial measurements of serum glucose were within normal limits. A blood smear showed hypochromic microcytic anemia. Widal, Wright, and tuberculin tests were negative.
Abdominal and pelvic ultrasonography was normal. An upper gastrointestinal endoscopy revealed erosions in the fundus and body of the stomach. A chest X-ray showed increased cardiothoracic ratio (Fig. ). An echocardiogram indicated dilated left ventricle (left ventricular dimensions were 55 × 44 mm), decreased fractional shortening (Fs; 13%), an ejection fraction (EF) of 26%, paradoxical septal movements, pulmonary blood flow of 0.7 m/second, and grade 2 mitral valve insufficiency. The right chambers were within normal range.
Urine culture revealed growth of Klebsiella species. Voiding cystourethrogram revealed no abnormal findings.
Based on her physical examination, she was judged to be euvolemic. To correct the euvolemic hyponatremia, water intake was restricted to 75% of the calculated daily need. Despite this, hyponatremia did not resolve. A cardiac ultrasound suggested dilated cardiomyopathy so cardiomyopathy management protocol (digoxin, furosemide, spironolactone, and captopril) was initiated with no remarkable improvement. She was also started on trimethoprim/sulfamethoxazole for the urinary tract infection until urine culture became negative.
Her history, along with the physical examination findings and laboratory evaluation suggested adrenal insufficiency. To confirm this, she underwent tests for the adrenal cortex function and the results were as following: random serum cortisol was 4.25 mcg/dL, adrenocorticotropic hormone (ACTH) 1500 pg/ml, and 17-hydroxyprogesterone 0.7 ng/ml. Hyponatremia, low cortisol, and high ACTH along with her symptoms suggested primary adrenal cortex insufficiency. She was treated with 100 mg/m2 intravenously administered hydrocortisone which was gradually reduced to 20 mg/m2 orally administered hydrocortisone before discharge. Remarkable improvement was noted within days of starting treatment. A heart echocardiogram before discharge showed considerable improvement (dimensions were 53 × 42 mm, EF 42.6%, and Fs 21.2%; Fig. ). After 2 weeks of hospitalization, she was discharged from our hospital on orally administered 20 mg/m2 hydrocortisone.
During the 6 months after discharge, she was followed-up to observe the clinical, laboratory, and radiologic improvements. Serial echocardiograms showed gradual restoration of cardiac function to near-normal status (EF 50% and FS 26%).
There were no signs of chronic mucocutaneous candidiasis or autoimmune hypoparathyroidism which, if present, would suggest autoimmune polyglandular syndrome type 1 (APS1). Since autoimmune polyglandular syndromes, congenital adrenal hyperplasia, bleeding into the adrenals, and tuberculosis were excluded and she had no history of glucocorticoid therapy, the cause of her adrenal insufficiency is mostly autoimmune. Immunologic tests to confirm this were not available.
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pmc-6247622-1
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In October 2013, a 78-year-old woman was admitted to our Oncology Unit because of the development of costal pain and cough. A chest CT-scan showed a complete atelectasis of the left lung inferior lobe, a solitary nodule in apex segment of the upper right lung lobe, the presence of multiple small nodules in basal posterior segments of the right lung lobe as well as in the middle and upper left lung lobes, and a left pleural effusion (Fig. a). She had a good performance status (PS) (ECOG PS = 0). Her prior medical history was remarkable for surgical excisions of two nodular BCCs of the trunk (0.5 × 0.3 cm and 0.6 × 0.7 cm, respectively, both without perivascular and perineural invasion) in 2003. In addition, she was an active smoker (40 packs/year).
In order to complete the pathological and clinical staging, the patient received a whole-body 18FDG-PET/CT and a bronchoscopy with a biopsy of the left lung lesion. The 18FDG-PET/CT showed a high metabolic activity of the lesion in the left lung, whereas the other lesions did not show any metabolic activity. The pathological examination demonstrated the diagnosis of lung squamous cell carcinoma (p63+, TTF-1-). She was staged as a stage IV NSCLC (according to TNM staging 7th edition).
Because of her good clinical conditions, the patient was treated with six cycles of chemotherapy with gemcitabine (1250 mg/m2), on days 1 and 8, plus cisplatin (75 mg/m2) on day 8, every 21 days. In May 2014, whole-body CT scan showed a partial response (PR) (according to RECIST 1.1 criteria) following six cycles of chemotherapy with 90% reduction of the hilar lesion and disappearance of the pleural effusion (Fig. b). Following multidisciplinary discussion, the patient received a consolidative radiotherapy treatment on the residual disease, obtaining a stable disease (SD) for an additional 8 months. However, in April 2015, a whole-body CT scan showed a progression of disease (PD) with an increased diameter of the left hilar lesion and the appearance of several hilar lymph nodes (Fig. c). Thus the patient received a second-line chemotherapy with 6 cycles of docetaxel (75 mg/m2) every 21 days, obtaining a SD. Unfortunately, in January 2016, a whole-body CT scan showed a PD with an increase of the pulmonary hilar lesion associated with atelectasis of the inferior left lobar bronchus and several pathological mediastinal lymph nodes (Fig. d). In order to reanalyze tumor histology and the molecular profile, we decided to perform a re-biopsy of the left lung lesion. Pathological examination confirmed the diagnosis of lung squamous cell carcinoma. No targetable oncogenic alterations (EGFR mutations, ALK/ROS-1 rearrangements and BRAF mutations) were detected. PD-L1 expression on tumor cells was scored as > 1% on tumor proportional score (TPS).
Based on these results, we decided to start a third-line treatment with the anti-PD-1 mAb nivolumab at the dose of 3 mg/kg, every 14 days. In February 2016, the patient started the administration of nivolumab. In May 2016, a CT scan showed a SD (Fig. e) which was confirmed in successive restaging of the disease (Fig. f). Following 18 cycles of nivolumab treatment (in January 2017), in a good performance status and without experience of any immune-related adverse event, the patient developed an ulcerated lesion (diameter = 1.0 × 1.4 cm) localized at the right ala of nose (Fig. a). While she was on nivolumab treatment, in February 2017, she underwent an excisional skin biopsy. Histological examination of the lesion showed an ulcerated nodular BCC. Perivascular and perineural invasions were not detected. Surgical tumor margins were negative. Immunohistochemical (IHC) staining of tumor showed a PD-L1 expression < 1%, on both tumor cells and immune cells, and lack of human leukocyte antigen (HLA) class I and β2-microglobulin (β2m) expression on tumor cells (Fig. ). In addition, a limited number of T cells (CD3+) was present in the tumor microenvironment, with a higher number of regulatory T cells (Foxp3+) and macrophages (CD11b+) as compared to activated cytotoxic T cells (CD8+/ Granzyme B+) (Fig. ). After two months, while still being treated with nivolumab, the patient relapsed with a new BCC lesion in the same region of the previous surgical excision (Fig. b). A new surgical excision was performed and the pathological examination confirmed the diagnosis of a relapsed BCC with same pathological characteristics.
At present, the patient is still being treated with nivolumab. She has received 46 cycles of nivolumab. Sustained stable disease control is still ongoing for metastatic NSCLC. She is in good health conditions. No treatment-related toxicities have been observed. No additional BCC relapses have been detected so far.
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pmc-6247625-1
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53-year-old man underwent abdominal ultrasonography (USG) during investigation for bilateral renal nephrolithiasis. The patient had normal blood biochemistry, and had no cardiovascular or hormonal disorder. He had been operated 17 years earlier to repair his extrophic bladder by creating an Indiana pouch. His weight was 85 kg.s with a body mass index of 28.7 kg/m2. USG showed a hyperechogenic lesion at the fat intensity filling out right renal sinus completely. Computerized tomography scan confirmed the presence of a fatty mass that extended from the renal sinus down to the pelvis cuffing the right ureter throughout its entire length with severe hydronephrosis. Left kidney was unremarkable except a small scar at the upper pole. The presence of high density regions inside the lesion imparted a suspicion for liposarcoma. The resection of the mass with right radical nephroureterectomy was performed.
Macroscopically, the tumor was 16x13x6 cm in size, fatty in appearance marbled with irregular whitish solid areas or fibrosis (Fig. ). Entrapped ureter was stenotic proximally and distally, but dilated at its middle part. Kidney was hydronephrotic with thinned out atrophic parenchyma. A few small stones were detected inside the collecting system. Microscopic examination of the tumor showed mature fat tissue which was devoid of normal architecture owing to expanded interstitial spaces either because of intense edema (Fig. ) or irregular fibrous streaks (Fig. ). Fibrous septa between fat lobules contained mildly increased numbers of stromal fibroblasts, fine collagen, vascular proliferation, multifocal lymphocytic infiltration, occasional lymphoid follicles and foamy histiocytes (Fig. ). Plasma cells were rare, and immunohistochemistry showed only < 2% IgG4+/ IgG+ plasma cell ratio. There were also scattered smooth muscle bundles usually in close association with vessels. Ectatic branching lymphatic channels were not noted. There were neither lipoblasts nor significant cellular atypia. A few scattered fibroblasts carried multilobulated large nuclei which were a bit worrisome (Fig. ), but these were rare and displayed regular chromatin distribution without hyperchromasia, thus thought to be reactive rather than neoplastic. Additionally, the immunohistochemistry for MDM2 and CDK4 gave negative results (insets-Fig. ) as well as stains for pan-keratins, HMB-45 and melan-A. Fluorescent in-situ hybridization analysis did not show MDM2 amplification. The findings were found compatible with MLL. The most possible predisposing factor in this current case appears to be operational trauma which occurred 17 years ago. He did not have lymphedema in the scrotum, legs, abdominal wall, or in the other regions of the body. The patient has stayed recurrence-free for the past 5 years after diagnosis.
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pmc-6247633-1
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A 62-year-old Japanese male with SCA6 was admitted to our hospital due to exacerbation of ataxia. He developed diplopia and body sway in his 30s. These symptoms gradually progressed, and gait disturbance and dysarthria appeared in his 50s. At age 61 years, he was genetically diagnosed as SCA6. He could walk by himself with bilateral Lofstrand crutches, but had recently begun to fall frequently. He had a family history of SCA6 in his mother and 2 siblings. None of them could walk by themselves and were using a wheelchair. Clinical examination revealed truncal and limb ataxia, dysarthria, horizontal gaze-evoked nystagmus, and slight vertical misalignment (slight restriction of upper ocular movement of the left eyeball) in a neurological confrontation test. The misalignment was clearer for the upper-right gaze (Fig. ). He had no cognitive dysfunction, his fundus was normal and he had no low vision.
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