Mrs. A. A, a 40-year-old Para 2-+-0 with no living child, (last child birth was 3 years and last menstrual period was 2 weeks prior to presentation) was seen at gynecological clinic with a history of abdominal distension of 3 years duration. The distension started gradually in the suprapubic region: - it later progressed to involve the entire abdomen. There was no change in the menstrual period and cycle. There was no change in urinary or bowel habit. There was no vomiting, cough, jaundice or abnormal vaginal bleeding. There was associated with mild abdominal discomfort and weight loss. She had been taking herbal medications since the onset of the above problem. When the mass became very large, it prevented the patient from carrying out her normal activities. The patient sought medical care at Awka General Hospital where a diagnosis of ovarian tumor was made. She was subsequently referred to Nnamdi Azikiwe University Teaching Hospital, Nnewi for expert management. She attained the age of menarche at 14 years. She was neither aware of Pap smear nor contraceptives. The past medical history was not contributory. There was no family history of such ailment. She was the third wife of her husband who was a farmer. She did not take alcohol or tobacco in any form.
On examination, she was chronically ill looking and pale. She was neither jaundice nor febrile. The pulse rate, blood pressure, and temperature were normal. Her body weight and height were 63 kg, and 160 cm, respectively. The cardiovascular and respiratory systems were normal. Abdominal examination revealed grossly distended abdomen, which was tense and visible veins [Figures and ]. The abdominal circumference was 126 cm measured at the level of the umbilicus. The liver, spleen and kidneys were difficult to assess due to the tensed abdomen. On vaginal examination, the vulva, vaginal and cervix appeared normal. The uterine size and adnexae were also difficult to assess because of the tensed abdomen. Abdomino-pelvic ultrasound revealed huge well circumscribed cystic mass with internal echoes arising from the pelvis. There were echogenic crystal-like solid components. The mass extended from the pelvis to the epigastrium and bowel loops were displaced posteriorly. There was no ascites. The liver, spleen and kidneys were normal from ultrasonography. Huge ovarian mass was a provisional diagnosis. The uterus was obscured by the cyst and could not be visualized; however, it appeared normal on transvaginal ultrasound. The urinalysis, completed blood count, liver function test, blood urea nitrogen, electrolyte and creatinine were normal. Her blood group was B Rhesus D- negative. She was admitted and worked up for laparotomy.
She underwent exploratory laparotomy on May 28, 2011 via low midline incision with upward extension up to about 6 cm below the xiphisternum. After laparotomy, a large tense smooth surfaced cystic mass that obliterated the peritoneal cavity was seen. There were no ascites. The intracystic fluid was drained through a small hole made on the mass. Approximate 20 L of straw colored thick fluid with debris was drained. Attempt to remove the mass with an intact capsule failed because it was adherent to the anterior abdominal wall and to loops of bowel posteriorly []. The cyst wall was eventually separated from the anterior abdominal wall by blunt dissection. The mass originated from the right ovary. It contained some debris. The left tube and ovary were normal. Ovarian cyst was removed by ipsilateral salpingo-oophorectomy. Estimated blood loss was 700 ml; two units of blood were transfused intraoperatively. The pathologists confirmed Mucinous cystadenoma of the right ovary was later confirmed from histopathology The post- operative period was uneventful and she was discharged on the 9th post-operative day.
She was followed up for 1 year and did not develop any symptom suggestive of pseudomyxoma peritonei. She was later lost to follow up.


A 10-year-old girl born out of second degree consanguineous marriage presented with complaints of regression of both motor and mental milestones from the age of 4 years. She was born naturally at full term followed by mild delay in milestones; however, she was fully ambulant and communicating in sentences by the age of 3. Then it was noticed that she developed difficulty in walking in the form of abnormal posturing of the trunk which aggravated during attempted motor tasks of the limbs and exertion. Later she developed plantar flexed posture at both ankles, dystonic posturing and choreiform movements of both upper limbs followed by self-mutilating behavior in the form of biting at the base of her thumb, biting the lips and the tongue. Later she developed difficulty in chewing, swallowing and became bed bound. She could comprehend simple commands and was communicating through sounds and gestures which her mother could comprehend.
Examination showed an emaciated patient. Her head circumference was 42 cm, weight 11 kg and height 102 cm. She was able to understand simple commands. Examination of her lips showed ulcerated, swollen lower and upper lips []. Several parts of the lower lips were missing. The ulcers were showing yellowish slough with areas of bleed. Her angle of the mouth showed ulceration and pigmentation. Minor ulcerations were also seen over the tongue and two of her incisors were missing in the lower jaw. She was in ophisthotonic posture and the right thigh had crossed over to the left thigh with the right leg placed below the left knee []. Any attempt to move the right leg produced severe pain. Examination of her nervous system showed pallor of both optic discs. She had orofacial dystonia and apraxia for swallowing. There was retrocollis, opisthotonus of the trunk [] with partial flexion at elbow, wrist, hip and knee. Choreiform movements were seen in both upper limbs. Her reflexes were well elicited and plantars were extensor.
She was investigated with blood tests and imaging. Her liver enzymes were elevated: Alanine transaminase was 173 U/l and aspartate transaminase was 534 U/l (normal value 8–50 U/L). Lactate dehydrogenase levels were elevated at 1208 U/l (normal < 248 U/l). Serum ceruloplasmin levels were 37 mg/dl (normal 20-35 mg/dl). Her peripheral smear showed normocytic to microcytic hypochromic blood picture. Her ferritin levels were in the lower limit of normal. There was no KF ring. Serum uric acid, serum copper and 24 hour urinary copper were normal. Urine screening for abnormal metabolites and tandem mass spectroscopy for inborn errors of metabolism was non-contributory. Her hip X-ray showed posterior dislocation of right hip []. Abdominal ultrasound showed coarseecho texture of liver suggestive of chronic liver disease. Cardiac evaluation was normal. Magnetic resonance imaging of Brain showed isointensity of the basal ganglia structures in T1-weighted images, hypointensity in the anteromedial putamen in T2-weighted images, and fluid attenuated inversion recovery (FLAIR) sequence. Susceptibility Weighted (SW) sequences showed hypointensity in the putamen suggestive of iron deposition[] [Figures -] Quantification of iron could not be done due to technical reasons. Genetic testing could not be carried out because of financial constraints.
The patient was treated as follows: Ulcerations in the lips healed with local applications and antibiotics. Her general condition improved with Ryle's tube feeding. Endoscopic gastrostomy was planned but parents were not willing for the same. Considerable improvement in dystonia occurred with oral trihexyphenidate and baclofen. Orthopedic consultation was done and suggested surgery after good control of dystonia.


A 48-year-old housewife presented to us with a painful solid mass over her upper back present for the preceding 2 years. Starting as a pea-size swelling, it increased gradually in size and became more painful. There was no history of prior trauma or any discharge from the lesion [].
On examination, there was a well-defined, very tender, yellowish, hard lesion of about 3 cm diameter, having a smooth irregular surface located over the midline in the upper back. It was not fixed to underlying tissue. There was no regional lymphadenopathy and no other mucocutaneous findings. Systemic review was non-contributory.
Fine-needle aspiration cytology (FNAC) showed large round to oval cells with vesicular nuclei. Few binucleated cells were embedded in abundant proteinaceous material. Histology showed sheets of large polygonal cells infiltrating the dermis. The cells were characterized by abundant, coarsely granular and amphophilic cytoplasm; with eccentrically located small vesiculated nuclei and prominent nucleoli. There was neither any mitotic figure nor spindling of the lesional cells [Figures and ].
Routine laboratory tests, blood biochemistry panel and imaging studies were within normal limits. Immunohistochemistry was not done due to local unavailability and financial constraints. Based on the clinical and histological findings, a diagnosis of GCT was made.


A 71-year-old male was transferred to our hospital with severe headache. A computed tomography (CT) scan showed subarachnoid hemorrhage (SAH), the distribution of which was dominant in the left side and relatively localized in the posterior fossa []. CTA demonstrated a 4 mm aneurysm with blebs in the direction to the left side in the right anterior choroidal artery (AChA) []. Based on a magnetic resonance image performed at a previous hospital, the AChA aneurysm was thought to be a ruptured aneurysm []. The aneurysm was surgically explored, but was found to be an unruptured aneurysm. DSA and subsequent 3DRA were performed. A small aneurysm measuring 1 mm in maximum diameter was depicted with 3DRA at the meatal segment of the left AICA []. The AICA aneurysm was not noted by 2DDSA []. The aneurysm was completely clipped, preserving the AICA main trunk, but the internal auditory artery that divided from the aneurysmal dome was difficult to preserve [Figure and ]. The patient has improved without any complications due to vasospasm, except for postoperative deafness on the left side.


A 20-year-old Vietnamese female had flu-like symptoms on April 11, 2013. Four days later, she visited a local hospital with exacerbated dyspnea. Her chest X-ray revealed diffuse infiltrations. She was clinically diagnosed with pneumonia due to influenza and administered oseltamivir (150 mg/day). She was immediately transferred to the ICU in our hospital. Her Glasgow score was 15 and she presented cyanosis, dyspnea, and tachycardia. Arterial gas analysis (AGA) showed severe hypoxemia (PaO2/FiO2 [ratio of arterial oxygen partial pressure to fractional inspired oxygen], 170) under 15 L/min of supplemental oxygen administered via a mask (Table ). A chest radiograph showed diffuse infiltrations (Fig. 1). Echocardiography was normal (ejection fraction/EF, 65%). She tested negative twice for influenza H1N1/09 virus by a real-time reverse-transcription polymerase chain reaction (RT-PCR) assay on a sputum sample, but later tested positive in a serological test. Bacteria were not detected in sputum or blood cultures.
She was diagnosed with ARDS. Ventilation support was initiated using biphasic positive airway pressure (BiPAP) along with the empirical treatments of oseltamivir and antibiotics (imipenem, levofloxacin). Twelve hours later, her PaO2/FiO2 ratio was 182. PMX-DHP was initiated and performed for 14 h on day 4 after the onset of symptoms. The PaO2/FiO2 improved to 375 on day 5 (Fig. ). On day 8, she was weaned from BiPAP to a nasal cannula. The PaO2/FiO2 improved to 380 with no oxygenation and her chest radiograph improved on day 9 (Fig. 2). She was discharged from hospital on day 12.


A 23-year-old Vietnamese male had flu-like symptoms on April 3, 2013. On day 3, he visited a local hospital because of exacerbated dyspnea. The chest radiograph revealed diffuse infiltrations. He was admitted to the local hospital and was treated with antibiotics (amikacin, ceftriaxone) with the diagnosis of bacterial pneumonia. His condition deteriorated. On day 9, he was transferred to our ICU and his Glasgow score was 15. AGA (under BiPAP) showed severe hypoxemia (PaO2/FiO2 of 89; Table ). A chest radiograph revealed diffuse infiltrations (Fig. 1). Echocardiography was normal (EF, 65%). Treatment with oseltamivir (300 mg/day) was initiated based on the possibility of influenza infection, along with broad spectrum antibiotics (imipenem, levofloxacin). On day 10, the PaO2/FiO2 had dropped to 78. PMX-DHP was initiated and performed three times at 8-h intervals (Fig. ). He also received continuous veno-venous hemofiltration (five times) as a non-renal indication between PMX-DHP treatments. Volume control ventilation was also initiated. On day 11, the PaO2/FiO2 had improved to 166. On day 12, it was 222, but on day 13, it had dropped to 128 (Fig. ). On day 14, bacterial infection was detected in blood cultures. The chest radiograph showed no improvement (Fig. 2). On day 16, the PaO2/FiO2 had dropped further to 68, and on day 18, the patient died of respiratory failure. A positive result for influenza H1N1/09 virus by RT-PCR on a sputum sample was obtained during ICU admission.


A man in his sixties was referred for evaluation of biopsy proven GF []. He had a known history of epilepsy. The lesion was first noted 15 years prior when a persistent, erythematous, indurated plaque appeared with associated telangiectasia over the right side of his forehead. Histopathologic findings were consistent with granuloma faciale showing a dense mixed infiltrate within the dermis, comprising of plasma cells, eosinophils, neutrophils and macrophages. Findings were consistent with a vasculitis, without fibrinoid necrosis. No defined Grenz zone was noted [Figures and ].
GF is notoriously difficult to treat. Multiple modalities have been used in the past with variable success including single therapy or combinations of colchicine, dapsone, antimalarials, gold injections, isoniazid, clofazimine, topical psoralen with UVA, corticosteroids, cryosurgery and various laser therapies.[] This patient had been treated with a number of these topical therapies prior to referral to our centre, each with poor results.
The patient was initially commenced on topical calcineurin antagonist (compounded tacrolimus 0.1%), before undergoing four treatments of vascular beam laser therapy at 595 nm and long pulsed 532 settings. This induced purpura, but again failed to produce a significant effect. Following this, a small improvement was noted with seven treatments of intralesional corticosteroid administration consisting of 5-10 mg of triamcinolone acetonide 10 mg/ml Kenacort-A 10 (KA10, Aspen Pharmaceuticals, St Leonards, Australia). Central atrophy was noted, but little change was seen at the edge of the lesion which remained thick and prominent []. The response was considered inadequate by both the patient and clinician. Systemic therapies were briefly considered but avoided in this patient, due to his comorbidities and long-term use of carbamazepine for epilepsy control.
The mild response of the lesion to intralesional steroids led to the idea of using intralesional 5-fluorouracil (5FU) as a steroid-sparing or replacement agent, in a similar way to the use of 5FU in hypertrophic and keloid scarring.[] 5FU acts in a similar way to a steroid agent in keloid and hypertrophic scars, without the unwanted atrophic effects.[] As first described by Fitzpatrick in the treatment of scars in 1999, an 80:20 mix of 80% 5FU (DBL Fluorouracil, 500 mg/10 ml, Hospira Australia Pty Ltd, Melbourne, Australia) and 20% KA10 was used to initiate treatment.[] This yielded a mixture in a 1 ml syringe of 0.8 ml 5FU and 0.2 ml KA10. When initially reported, the small amount of steroid was added to the 5FU to limit the pain of injection.[] The patient received intralesional KA10 and 5FU combination injections initially to the outer rim, and subsequently over the entire lesion. Various mixture quantities were used, ranging from 0.1 to 0.3 ml of KA10 given in combination with 0.7-0.95 ml of 5FU. These were given at intervals ranging from 2 weeks to 2 months according to patient availability and convenience.
The patient has now received a total of twenty injections over a period of more than 3 years. An excellent response has been noted and the patient is now able to tolerate long treatment-free periods between 9 and 12 months []. The small volume of KA10 relative to 5FU in the mixture suggests the response can primarily be attributed to the inclusion of 5FU, as the patient had not adequately responded to a pure steroid injection at higher concentration received initially.


A 60-year-old lady with no co-morbid illnesses presented to our center with a painless swelling in the right side of the upper neck of 6 months duration. Her past history included treatment for carcinoma right breast at our center 7 years prior. (T3N1M0 for which she received multimodality treatment consisting of surgery, chemotherapy and radiotherapy) The histopathology of the mastectomy specimen revealed an infiltrating ductal carcinoma and mucinous carcinoma. The tumor cells showed strong positivity for estrogen receptor (ER) and progesterone receptor (PR) and the patient had received adjuvant aromatase inhibitor (letrozole 2.5 mg) for 5 years and was on regular follow-up.
Clinical examination revealed a large well circumscribed, firm swelling measuring 6 cm × 5 cm in the right side of the upper neck. Examination of the cervical lymph nodes and the upper aero-digestive tract was normal and so were the examinations of the left breast, right chest wall, both axilla and supraclavicular fossa. Fine needle aspiration cytology from the mass was reported as poorly differentiated carcinoma.
A fluorine-18 fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) done as a part of the staging work up revealed an well enhancing irregular lobulated soft tissue mass measuring 6 cm × 5 cm × 4 cm arising from the right PPS in relation to the deep lobe of the right parotid gland, extending from the level of the cervical vertebra C1 to C5, causing mass effect on the major neck vessels, that is, infiltrating the right internal jugular vein and displacing the right carotid vessels without displacing it (SUVmax 9.1). There was no other significant uptake on the PET-CT [Figure –].
The provisional diagnosis was that of a malignant salivary gland carcinoma involving the PPS. A rare possibility of a parapharyngeal metastasis from breast carcinoma was also considered in view of the prior history of breast cancer.
It was felt that surgery would still be the treatment of choice even in the later scenario; considering the long disease free interval and the solitary site of metastasis. The patient was therefore planned for a definitive radical surgery. Intra-operatively the mass was dissected out from the surrounding structures and was removed en bloc along with the right internal jugular vein and the neck nodes; resulting in a formal radical neck dissection [Figure and ], The patient made an uneventful postoperative recovery. Microscopically, the tumor cells were arranged predominantly as nests and islands with few foci of tubule formation, separated by scanty stroma. They were round to polygonal, with moderate eosinophilic cytoplasm, vesicular nuclei with fine chromatin []. Increased mitosis and vascular invasion was also seen. On immunohistochemistry (IHC), the tumour cells showed positivity for PR, ER, [], pan-keratin and high molecular weight keratin []. The Ki-67 proliferative index was 50–60% []. Further the tumour cells were positive for the neuroendocrine markers CD56, neuron-specific enolase, synatophysin [], chromogranin []. The tumour cells showed negative reaction to C-kit, gross cystic disease fluid protein-15. Based on the histopathological and IHC findings, the tumour was finally diagnosed as metastatic carcinoma of breast with neuroendocrine differentiation.
The patient after a multidisciplinary board discussion was subsequently considered for external beam radiotherapy to the tumor bed and is further planned for a second line chemotherapy followed by hormonal therapy.


A 78-year-old male patient was admitted with a 5.5 cm-sized infrarenal abdominal aortic aneurysm (AAA) and early gastric cancer (EGC) detected during routine screening. His medical history revealed hypertension controlled by medications and he had no previous operation history. After evaluation of the gastric cancer, treatment for AAA was performed first, followed by staged treatment for EGC. EVAR was performed with a GORE EXCLUDER AAA Endoprosthesis (WL Gore & Associates, Newark, DE, USA) and distal laparoscopic gastrectomy was performed 12 days later. Both procedures were uneventful.
On the 18th post operative day (POD) from gastrectomy, the patient pre sented with intermittent fever and lower abdominal tenderness. Laboratory results showed leukocytosis (18.5×109/L) and increased C-reactive protein levels (13.4 mg/dL). Blood cultures revealed methicillin-sensitive Staphylococcus aureus bacteremia. The patient underwent computed tomography (CT) scan that revealed perigraft fluid collection around the right iliac limb graft (). Echocardiography also revealed mitral valve vegetation which was consistent with infective endocarditis. After consultation with the infectious disease specialist, stent graft removal was decided in order to control the bacteremia. Stent graft removal was performed on the 49th POD after EVAR (37th POD after gastrectomy) with in situ reconstruction using autogenous SFV.
After long midline laparotomy, exploration of the abdominal cavity revealed relatively normal findings without evidence of severe adhesion, despite having undergone a previous operation. Through a retroperitoneal approach, the previous aneurysm sac was exposed and opened. The stent graft, aneurysm sac and periaortic tissues were excised entirely. Fortunately, the graft could be removed easily without the need for modified explantation techniques such as the use of a syringe []. The proximal right iliac artery was resected and the stump was ligated in a double-layered fashion. A drain was inserted along the right iliac artery territory to prevent progression of infection in the right groin sinus.
After removal of the infected stent graft, the left SFV was harvested for a length of 20 cm with preservation of the great saphenous vein. The venous graft was anastomosed end-to-end with the aorta in a non-reversed fashion. After restoration of aortic flow, valvulotomy was performed (–). The graft was sutured distally with the left common iliac artery in end-to-end fashion. Finally, crossover femoro-femoral bypass with a polytetrafluoroethylene (PTFE) graft was performed with subcutaneous tunneling through a clean subcutaneous fat layer. Bilateral pulses were strongly palpable after the whole procedure.
There were no immediate complications during hospital stay. One month after reconstruction, laboratory findings were normalized and there was no further bacterial growth in blood cultures, while CT scan and echocardiography showed improved state of infection and infective endocarditis (). After management of residual infection and infective endocarditis, the patient was discharged on the 46th POD after aortic reconstruction without any complications.


A 60-year-old woman attended to obstetrics and gynecology outpatient department of our medical college with multiple asymptomatic wart-like lesions over vulva and mons pubis. There was no history of burning, pruritus or associated pain. According to the patient, the lesions had developed since 4-5 years. There was no history of pelvic surgery, prior chemotherapy, gastrointestinal disease or tuberculosis. On examination, multiple warty lesions are seen ranging from 3-10 mm in size in the vulva and mons pubis. Abdominal and pelvic ultrasonography (USG) were done and were normal. Routine blood examination was normal except mild leucocytosis. With a clinical differential diagnosis of wart, molluscum contagiosum and lymphangioma, biopsy was taken from lesion and sent to us for histopathological evaluation.
Hisopathological examination revealed acanthosis and hyperkeratosis of epidermis with numerous dilated lymphatic channels containing eosinophilic proteinous material in the epidermis and papillary dermis []. Thus a diagnosis of LC was confirmed. Patient underwent surgical procedure with removal of vulva and involved area with 3 cm in depth. After surgery, the sample was sent to pathology department for further examination []. Histopathological examination once again confirmed the diagnosis of LC and the resected margins were clear. There was no evidence of malignancy. The patient was followed up for 1 year and there was no sign of recurrence during this period.


Here, the authors report a case of a 43-year-old Caucasian man with a medical history of
end-stage renal disease due to hypertensive nephropathy and a 4-year history of
hemodialysis. Prior to the initiation of hemodialysis, the patient underwent renal
transplantation and rejected the allograft after 5 years due to voluntary
discontinuation of immunosuppressive therapy. He was admitted to the hospital with
fever, malaise, altered mental state and chest pain. Physical examination revealed
cognitive impairment with incoherent speech, left arm weakness, exacerbated
osteotendinous reflexes, the presence of the Babinski reflex on the right side and three
episodes of complex partial seizures. He was medicated with valproic acid and fenitoin
for seizure control.
Relevant laboratory findings included creatinine 13.17mg/dL, urea 203 mg/dl, potassium
7.4mEq/L, leucocytosis 20,000/dL, C-reactive protein 411.5mg/L, troponin I 2.7ng/mL and
myoglobin 317IU/L.
An electrocardiogram showed ST segment elevation in DII, DIII, aVF, V2 and V3 with
upward concavity and depression of the PR segment in DII, V2 and V3.
Transthoracic echocardiography revealed normal left ventricular dimensions and systolic
function with no left atrial enlargement; furthermore, the right cavities were not
dilated, and the right ventricular systolic function was preserved. A mild pericardial
effusion with no evidence of vegetation in the valve leaflets was observed.
A computerized tomography scan of the brain revealed a right frontal
cortical-subcortical lesion with ischemic and hemorrhagic components (). Magnetic resonance imaging confirmed
multiple hemorrhagic lesions in both cerebral hemispheres (). A thoracoabdominal computerized tomography scan revealed
septic pulmonary embolus and a splenic infarction. The diagnostic hypothesis was IE, and
the patient underwent transesophageal echocardiography, which identified vegetations in
the aortic and tricuspid valves of 9.13mm and 11.73mm in size, respectively ( and ), with mild aortic regurgitation and moderate tricuspid regurgitation.
Empirical treatment with ceftriaxone, gentamicin and vancomycin was initiated. Blood
cultures were positive for methicillin-sensitive S. aureus, and the
empirical treatment was changed to flucloxacilin and gentamicin. Cerebral spinal fluid
was unremarkable, and its culture was sterile. The pericardial effusion became bulkier
and generated late diastolic inward motion of the right ventricular free wall and the
right atrium, which necessitated pericardiocentesis.
Despite the administration of effective antibiotic therapy, cerebrovascular embolic
events persisted with neurologic deterioration. The multidisciplinary team decided that
the patient should undergo valve replacement after the stabilization of the intracranial
hemorrhage. However, on the 8th day after hospitalization, the patient
entered cardiac arrest and was recovered with advanced life support. Transthoracic
echocardiography after resuscitation demonstrated an enlargement of the right cavities
and right ventricular hypocinesia, and the presumed diagnosis was a massive septic
pulmonary embolism. The patient re-entered cardiac arrest, and although fibrinolysis was
performed and advanced life support was maintained for 40 minutes, the patient died.


A 72-year-old female patient was admitted to our hospital for the evaluation and proper management of a pancreatic mass. Six months ago, she underwent a radical subtotal gastrectomy with gastroduodenostomy (Billroth I) due to early gastric cancer. This pancreatic mass was incidentally identified on a postoperative follow-up abdominal-pelvic computed tomography (CT) scan. Her body weight was 39 kg and the height was 144 cm (body mass index, 18.8). A physical examination finding was an upper midline abdominal skin incision in the abdomen, which was soft and flat without palpable mass. All routine blood laboratory tests including amylase and lipase were within normal ranges. Also tumor markers, carcinoembryonic antigen (CEA), carbohydrate antigen 19-9 (CA 19-9) and carbohydrate antigen 125 (CA 125) were within normal limits. In the CT scan, an approximately 2.5 cm-sized mass near the neck of the pancreas with distal duct dilatation and a parenchyma atrophic change abutting to the portal vein was identified. No lymph node enlargement or distant metastasis was detected ().The 2-deoxy-2-[18F]-fluoro-D-glucose (FDG) positron emission tomography (PET) showed approximately 2.6 cm-sized low-attenuating pancreatic mass in the body portion without significantly increased FDG uptake in any other organs (). Pancreaticobiliary magnetic resonance imaging with cholangiography showed about 2.5 cm-sized hypervascular, T2 low signal, fat-containing, well-defined mass in the neck area with distal parenchymal atrophy and duct dilatation (). The characteristics of the tumor seemed to be somewhat different from usual ductal adenocarcinoma of the pancreas, and serum AFP was checked. It was elevated to 2,254.1 IU/ml (reference range: 0-7.0 IU/ml).
The patient underwent a pancreaticoduodenectomy (PD). During the laparotomy, malignant ascites, peritoneal seeding or distant metastasis were not found, but severe peritoneal adhesions due to the previous radical subtotal gastrectomy were noted. The anatomic plane was obscured during the PD due to the previous lymph node dissection around the celiac axis, common hepatic artery and the proper hepatic artery. Therefore, a dissection between the neck of the pancreas and superior mesenteric vein-splenic vein-portal vein confluence was needed to identify the anatomic landmark (). As previous lymph node dissection around major vessels was conducted already, a standard lymph node dissection was performed (). Due to the previous gastroduodenostomy the reconstruction of gastrointestinal continuity was easy following the PD. All procedures, pancreaiticojejunostomy, hepaticojejunostomy and gastrojejunostomy were completed in a usual manner.
An 1.9×1.7 cm-sized ill-defined lobulating tumor confined to the pancreas was revealed in the macroscopic specimen examination (). An AFP-producing ACC of the pancreas (T1 stage) was classified based on the routine hematoxylin and eosin (H&E) staining and immunohistochemical staining ().
The postoperative recovery course was uneventful. The patient discharged 12 days postoperatively. For the potential possibility of application of chemotherapeutic agents in this rare case of pancreatic cancer, an ATP-CRA chemosensitivity test to anticancer drugs was performed (). The result showed cisplatin would potentially be more applicable than gemcitabine which is usually used in pancreatic cancer. Although the serum level of AFP had been dropped into normal range immediately after PD, it was rebounded exponentially 17 month postoperative (). The patient died due to carcinomatosis and pneumonia at 30 months after the surgery.


A 70-year-old female with diabetes, osteoporosis, hypertension and atrial fibrillation presented with advanced renal failure, progressive anemia and signs of peripheral polyneuropathy. Due to reversible brachio-facial cerebral ischemia in 2000 and chronic atrial fibrillation, she was on permanent warfarin therapy. On physical examination, she was hypertensive with no overt signs of fluid overload. Retrospective chart analysis revealed a rise in serum creatinine from 1.48 mg/dl (estimated glomerular filtration rate [eGFR] of 35 ml/min/1.73 m2 body surface area [BSA]) in November 2008 to 5.68 mg/dl (eGFR of 7 ml/min/1.73 m2 BSA) on admission in February 2010. The patient was anemic but normocalcemic (hemoglobin [Hb] 9.5 g/dl, calcium 2.41 mmol/l). Ultrasound showed normally sized kidneys with increased reflexivity. Serum protein electrophoresis disclosed IgA and IgG deficiency with IgM levels within normal limits. 24 h urine analysis showed a urine volume 850 cc and a proteinuria of 1400 mg/day. Immunofixation of the urine disclosed monoclonal kappa light chains of 2.6 mg/dl. The serum kappa to lambda free light chains (FLC) ratio was calculated at 288, well above proposed normal limits of 0.37–3.1 in patients with acute kidney injury without multiple myeloma. Bone marrow aspiration showed 30–40% infiltration of bone marrow lacunae by typical plasmacytoma cell nests. LCMM expressing kappa light chains was thus diagnosed. X-ray evaluation did not show typical signs of osteolytic lesions but diffuse osteoporosis. Cardiac magnetic resonance imaging could not be performed as the patient had a pacemaker implanted to treat sick-sinus-syndrome but echocardiographic evaluation was in line with a mild form of AL-induced amyloid deposition disease. Due to the therapy with warfarin concomitant with a prolongation in bleeding time and clinically manifest nasal bleeding, a renal biopsy was not performed. To treat renal failure, a double-lumen central vein catheter was placed in the right jugular vein and chronic hemodiafiltration (HDF) was initiated. At the same time, standard chemotherapy with melphalan and dexamethasone with concomitant application of bisphosphonates during each chemotherapy cycle was started. Daily HDF (except on Sunday) was carried out from the 5th to 12th of February 2010, followed by standard thrice weekly HDF therapy. Kappa light chain reduction ratios, as well as different other clearance parameters, were calculated for a total of 13 treatment sessions [Tables -].
Although urgent daily HDF and chemotherapy were started, the patient did not recover renal function on follow-up. Since February 2010, eight cycles of chemotherapy have been given with a total dosage of 70 mg of melphalan. Following the initial therapy, the patient was hemodialyzed 3 times/week. Laboratory analysis was as follows: Creatinine 7.73 mg/dl (eGFR of 6 ml/min/1.73 m2 BSA), Hb of 12.7 g/dl while on chronic erythropoietin medication (darbepoetin alpha 20 μg/week), calcium 2.34 mmol/l and ß2-microglobulin 17,200 μg/l. Repeat bone marrow aspiration after the fifth cycle of chemotherapy showed a reduction of myeloma infiltration to 10%. After 3 years, the patient died in the immediate postoperative period after suffering from an acute occlusion of the distal aorta (Leriche syndrome).


A 17-year-old boy visited Kyung Hee University Medical Center because of a painless, enlarging left scrotal mass. Scrotal ultrasonography revealed an approximately 6.6-cm-sized left testis with a partially cystic portion. Abdominopelvic and chest computed tomography (CT) showed enlarged, conglomerated left paraaortic lymph nodes and right retrocrural lymph nodes (). The patient underwent left radical orchiectomy. The pathologic report demonstrated a mixed germ cell tumor (immature teratoma 65%, embryonal carcinoma 15%, yolk sac tumor 10%, seminoma 5%, and choriocarcinoma 5%). Tumor markers after orchiectomy were significantly elevated: alpha-fetoprotein, 169.5 ng/mL; beta-human chorionic gonadotrophin, 23,245.16 mIU/mL; and lactate dehydrogenase, 644 U/L. After thorough counseling about treatment for multiple enlarged lymph nodes, the patient decided to receive chemotherapy with bleomycin, etoposide, and cisplatin. Three cycles of chemotherapy were administered, and the levels of all tumor markers decreased; however, the follow-up abdominopelvic CT scan showed increased lymph node size (). Therefore, we decided to perform R-RPLND for resection of the remnant mass after chemotherapy.
The patient was positioned in a right lateral decubitus position with the left side up and the bed flexed. After pneumoperitoneum was established by use of a Veress needle, a 12-mm camera port was placed at the umbilicus and five more trocars were used in the abdomen: three for robotic arms and two for the assistant (). The robot was docked at the patient's left side, and the assistant was positioned on the patient's right side ().
A left-modified template dissection was planned. The procedure was started with an incision at the line of Toldt. After the colon dropped medially, ligation of the gonadal vein was performed, and the spermatic vessel was dissected. The left ureter was easily identified and was isolated with a nelaton catheter. To prevent ureteral injury during dissection of lymph nodes, the ureter was retracted by the third arm of the robot. Paraaortic lymph nodes were dissected from the level of the common iliac artery to the root of the left renal artery along the lateral margin of the aorta (). Dissection of lymph nodes was a difficult and time-consuming process because of severe adhesion between the lymph nodes and great vessels as a result of chemotherapy. Postganglionic sympathetic fibers were detected and preserved to maintain ejaculatory function. Multiple aorto-caval lymph nodes were also observed () and were all dissected carefully by using Hem-o-lok clips and small metal clips. The skeletonized aorta and inferior vena cava were observed (). All of the resected lymph nodes were placed in an EndoCatch bag. A drain tube and a Foley catheter were inserted, and the surgery was completed.
The total operative time, including console time, was 420 minutes. There were no postoperative complications. The Foley catheter was removed on the first postoperative day, and the patient was discharged on the fourth postoperative day. Pathology revealed a count of 20 lymph nodes, 2 of which showed a metastatic immature teratoma. The patient received two cycles of chemotherapy with etoposide and cisplatin. At the time of this writing, his laboratory tests were within normal limits, and there was no evidence of metastasis on radiologic examination (). Furthermore, the patient had not complained of a decreased amount of semen or a dry ejaculation.


An 11-month old boy was addressed to our cardiological outpatient clinic for investigation of a heart murmur recently discovered by his pediatrician.
Personal and familial history was uneventful. He was born by elective caesarian section and had normal post-natal adaptation. He showed excellent development without any sign of cardiac failure or neurological alteration.
Clinical examination: a 2/6 systolo-diastolic murmur with punctum maximum over the 2nd left intercostal space and the left supra-clavicular region was auscultated. The neurological examination was completely normal.
Complementary examinations:
Echocardiography: ventricular size and function were normal as were flow patterns over AV- and semi-lunar valves. A patent ductus arteriosus could not be identified. Instead of that, a small collateral vessel arising from the distal aortic arch was detected but could not be identified further.
Cervico-thoracic angio-computer tomogram and angio-magnetic resonance imaging (MRI) demonstrated the presence of a vascular malformation. It consisted of an abnormal dilated left cervico-vertebral artery originating from the proximal segment of the descending aorta and feeding a cervico-thoracic paravertebral vascular network which further extended through a slightly dilated intervertebral foramen and connected with slightly dilated veins belonging to the anterior epidural venous plexus. No further dural or subdural vascular abnormalities were objectivized as well as no bone erosion ( and 2).
On follow-up MRI’s, the stability of the vascular malformation was demonstrated as well as the strict epidural extension. MRI indeed established the absence of intradural venous congestion, medullary compression or edema. Angio-MRI was repeated at 24 and 36 months of age.
Interventional fistula embolization is scheduled.


A 16-month-old boy was addressed to our consultation because of the presence of a vascular murmur in the right paravertebral lower thoracic area.
Personal and familial history was uneventful.
Clinical examination: 2/6 systolo-diastolic murmur with punctum maximum over the left para-vertebral area of the dorso-lumbar region. Besides that, clinical examination was normal, in particular there was no pulse amplitude difference between upper and lower extremities. There was no sign of cardiac failure or neurological symptoms.
Complementary examinations:
Echocardiography: The cardiac structure and function were normal, in particular, aortic arch and descending aorta showed normal size and flow pattern.
Angio-MRI objectivized the presence of a dorsal paraspinal vascular malformation mainly fed by a right enlarged 10th intercostal artery prolonged by an ectasic intra-foraminal periradicular branch connected with enlarged epidural veins. Less enlarged 9th and 11th intercostal arteries seemed to feed the malformation as well (). The absence of dural or epidural vascular anomalies as well as the absence of medullary compression or edema was obvious.
Owing to the young age of the child, fistula embolization is differed under close follow-up examinations.


A 21-year-old woman with a family history of HME presented to a chiropractor with a 2-month history of bilateral hand numbness in the fourth and fifth digits and progressive neck and upper back pain. Plain radiographs of her neck identified a large thoracic lesion (), which prompted a neurosurgery consultation before any chiropractic manipulations were performed. The patient denied any loss of fine motor dexterity, bowel or bladder dysfunction, or gait difficulty. Physical examination revealed no weakness, sensory deficit, dysreflexia, or signs of myelopathy. Palpable bony protrusions were identified on her bilateral proximal metacarpals. A thoracic computed tomographic (CT) scan with bone windows showed a large expansile lesion with mature osseous architecture arising from the medial right aspect of the second and third ribs. The lesion enveloped the right T2 and T3 pedicles, with encroachment on the right T2-3 neural foramen causing approximately 50% spinal canal stenosis (). A thoracic magnetic resonance imaging (MRI) study showed displacement of the thecal sac and the development of a small syrinx at this level but no abnormal signal change within the spinal cord (not shown). Imaging also revealed similar smaller osseous lesions arising from the costovertebral joints at T9 and T10, the vertebral body at L2, and the right scapula (not shown).
Surgical decompression of the spinal canal was performed in a posterolateral manner via costotransversectomy. A semilunar skin incision was used with the arc oriented toward the right overlying the origin of the exostosis. Dissection was carried down until the right T2 and T3 costotransverse joints, transverse processes, and lamina were exposed. Hemilaminectomy of T2 and T3 and costotransversectomy of T3 were performed. The right T3 costal head, proximal 4 cm of rib, transverse process, pedicle, and posterolateral vertebral body were removed to obtain the appropriate tangential angle to expose the anterior compartment of the spinal canal.
The tumor arose from the T3 costal head and extended both ventrolaterally to abut the pleura and medially through the T2-3 neural foramen and into the spinal canal ( and ). A cartilaginous cap surrounded by mature osseous architecture was visualized within the mass. The semilunar incision enabled excellent exposure both to resect the cartilaginous cap and decompress the intervertebral foramen and spinal canal. The prominence of tumor was debulked from the region between the second and third rib heads to the intraspinal space by using a high-speed drill with diamond bur (). The thecal sac was markedly displaced towards the left. Adhesions to the thecal sac were detached carefully so the dura was not violated. Intraoperative ultrasound was used to confirm adequate decompression of the spinal cord. After the decompression, a mass of mature bone was left between the T2 and T3 vertebral bodies, but there was no appreciable cartilaginous portion of the tumor remaining.
Intraoperative thoracic manipulation did not reveal abnormal motion at the affected levels so instability was not suspected. The tumor was not resected further so spinal instrumentation and fusion could be avoided. The decision was made to close the incision and wait for the pathological diagnosis, which, if it was malignant tumor, would guide further surgical and/or medical treatment. Histological examination of the operative specimen, however, confirmed the diagnosis of osteochondroma. Thus, given the patient’s age and adequacy of the decompression, further osteochondroma removal and risk of spinal destabilization were not warranted.
The patient had an uneventful hospital course and was discharged on postoperative day 3. She developed no new neurological deficits and reported improvement in her pre-operative neck pain at her 1-year follow-up examination. Imaging obtained at 1, 3, and 5 years after the operation showed a stable 3×2-cm residual mass at T2-3 that remained unchanged in size on each successive study, and no new osteochondromas were identified (). Interval thoracic radiographs demonstrated normal anatomic alignment of the thoracic spine with no signs of instability.


A 21-year-old female patient reported to the department with a chief complaint of swelling in the maxillary right labial sulcus. The patient's medical history was noncontributory. Dental history revealed trauma to the anterior teeth, with avulsion of the maxillary left central incisor when the patient was 9 years old. There was also a history of trauma for a second time to the anterior teeth a year back. Intraoral clinical examination showed a soft and fluctuant swelling in the labial sulcus region of teeth no. 11 and no. 12. Mesial tilting of tooth no. 11 was evident due to missing tooth no. 21. Teeth no. 11 and no. 12 were discolored and were nonresponsive to both electric and thermal pulp testing. Periodontal probing depths were within normal limits.
The preoperative radiograph displayed a large radiolucency, measuring approximately 13 mm × 11 mm apparently involving the apices of teeth no. 11 and no. 12. Tooth no. 11 revealed a large canal space with an open apex with ragged and irregular margins typical of external inflammatory resorption [].
Following the rubber dam application, access cavity preparations were done for both teeth no. 11 and no. 12. There was drainage of purulent exudate from the canal of tooth no. 11. Tooth no. 12 was found to be nonvital, but there was no evidence of exudate from the canal. A 23-gauge needle attached to a 5 ml syringe (Dispovan, Hindustan syringes and medical devices Ltd., India) was inserted into the root canal past the open apex, beyond into the periapical region. The purulent fluid was aspirated while simultaneous digital pressure was applied on the swelling in the labial sulcus. 3% sodium hypochlorite (Vensons India, Bangalore, India) was the irrigant used during instrumentation. Following working length determination, cleaning and shaping of both teeth no. 11 and no. 12 was carried out. Tooth no. 11 was instrumented minimally with a no. 60 H-file (Dentsply Maillefer, Ballaigues, Switzerland), while tooth no. 12 was instrumented with ProTaper NiTi rotary instruments (Dentsply Maillefer, Ballaigues, Switzerland). Digital pressure was applied in the labial vestibule while the canals were dried with paper points (Dentsply Maillefer, Ballaigues, Switzerland) and temporized with Cavit (ESPE, Seefeld, Germany).
At the next appointment after 3 days, a mild swelling was still evident in the labial sulcus. The aspiration through the root canal procedure was repeated. Since, the fluid was purulent in nature, it was decided to place a triple antibiotic paste of ciprofloxacin 500 mg (Ciplox-500, Cipla Ltd., Sikkim, India), metronidazole 400 mg (Aristogyl, Aristo Pharmaceuticals Pvt Ltd., Mumbai, India), and minocycline 100 mg (Minoz 100, Ranbaxy Laboratories Ltd., Solan, India), in tooth no. 11. When the drainage ceased, and there was no more aspirate, a creamy antibiotic paste was prepared and placed in the canal of tooth no. 11 with the help of a no. 55 K-reamer (Dentsply Maillefer, Ballaigues, Switzerland). The paste was restricted only till the canal orifice to minimize tooth discoloration. Calcium hydroxide (Ca-Excel, Ammdent, India) was placed as an intracanal medicament in tooth no. 12. After 1 week, there was no swelling evident in the labial sulcus and the patient was asymptomatic. Tooth no. 12 was obturated, while triple antibiotic paste in tooth no. 11 was kept for a further 1 week. At the next scheduled visit, the triple antibiotic paste was irrigated out of the canal. There was no evidence of any exudate from the canal. Calcium hydroxide was then used as the intracanal medicament, which was refreshed every 15 days. At the end of 2 months, there was a satisfactory decrease in size of the radiolucency and radiographic []. An apical barrier of mineral trioxide aggregate (Angelus, Londrina, Brazil) was then placed to a thickness of about 4 mm. Some amount of hard tissue formation was also evident on the distal apical root end of tooth no. 11, indicating cessation of active external inflammatory resorption []. Absorbable gelatin sponge (Gelfoam, Pfizer, USA) was carefully placed beyond the confines of the canal as an apical matrix. The mineral trioxide aggregate was mixed in a ratio of 3:1 and placed with the help of an amalgam carrier (Pulpdent, USA) and condensed into place with pluggers (Dentsply Maillefer, Ballaigues, Switzerland). A moist cotton pellet was then placed at the canal orifice, and the access cavity was temporized. After 48 h, the mineral trioxide aggregate was checked for complete set and the remainder of the root canal space was obturated with Gutta-percha (Dentsply Maillefer, Ballaigues, Switzerland) and AH plus sealer (Dentsply DeTrey, Konstanz, Germany). The access cavities of both teeth no. 11 and no. 12 were restored with composite resin (Tetri-N-Ceram, Ivoclar Vivadent, Liechtenstein). The recall radiograph after 1 year shows considerable healing of the periapical lesion []. The recall radiograph after 1 year and 6 months [] shows complete periapical healing and also the formation of a hard tissue layer over the mineral trioxide aggregate.


A 46-year-old man consulted for small patches of alopecia involving the sideburns and the beard area. Patient stated the disease had developed 3 years before and was diagnosed as alopecia areata. Treatment with intralesional and topical steroids did not produce regrowth. Clinical examination showed patchy alopecia of the beard and small atrophic patches of alopecia on sideburns []. Dermoscopy showed loss of follicular openings and thin peripilar casts []. A punch biopsy was obtained using the dermoscopy guided biopsy technique and was bisected as horizontal sections. It showed overall preserved follicular architecture with a focal area of follicular dropout and disappearance of the sebaceous glands. The follicular density was significantly reduced to five follicles (three terminal follicles and two intermediate size follicles) []. At the level of the isthmus, one follicle showed perifollicular fibrosis and a mild lichenoid infiltrate in perifollicular distribution []. Naked hair shafts were observed in the dermis within fibrotic tracts. These features were consistent with lichen planopilaris (LPP).


A 6-year-old girl, second child of a third-degree consanguineous marriage, presented with patchy hair loss since 18 months involving large areas of the scalp. She also suffered from eyelid defects since birth, abnormally wide eyes with narrow palpebral fissures and drooping eyelids, which was operated and diagnosed as a blepharophimosis-ptosis-epicanthus inversus syndrome (BPES).
On examination, there was a subtotal loss of scalp hair with no atrophy or scaling. Exclamation mark hairs were seen along the borders of patches. Body hairs were also sparse, but eyebrows and teeth were normal. The palpebral fissures were narrow; there was bilaterally symmetrical ptosis and telecanthus. Linear hypopigmented surgical scars were seen along canthi. Dermoscopic examination showed yellow dots and exclamatory mark hairs in the periphery and scalp biopsy revealed peribulbar lymphocytic infiltrate []. Karyotyping was performed to exclude trisomies, genetic analysis was offered but declined due to financial reasons.
She was started on topical immunotherapy with diphenylcyclopropenone (DPCP). Within 4 weeks of starting, re-growth of hairs was noted. Within 4 months, there was full thickness growth of hair covering entire scalp [Figures and ]. The treatment was tapered and stopped in 10 months. She is under follow-up since 1-month, and her hairs are continuing to grow with no abnormal shedding.


A 56-year-old man developed chronic otorrhea related to left tympanic membrane perforation. He was admitted for an elective left typanomastoidectomy and removal of the incus and malleus with tympanoplasty. His medical history included hypertension, dyslipidemia, gout, polycystic kidney disease and gastroesophageal reflux disease. His surgical history was significant for previous left tympanoplasty and inguinal hernia repair. His medications at the time of admission were rosuvastatin, domperidone, esomeprazole, allopurinol, irbesartan/hydrochlorothiazide and labetalol.
On the first day postoperatively the patient experienced a sudden drop in his level of consciousness accompanied by marked agitation, and required intubation. His Glasgow Coma Scale score was 9 (eyes 3, verbal 3, motor 3). There were no focal neurological signs, his pupils were symmetrical but sluggish to react and the fundi appeared normal. His blood glucose level was 9.0 mmol/L. He was subsequently transferred to the intensive care unit. A computed tomography scan of the patient’s head showed no structural abnormalities, no masses and no hematoma. Lumbar puncture was performed and revealed cloudy cerebrospinal fluid (CSF) with an elevated protein level (5.78 g/L), low glucose level (<1.0 mmol/L) and a leukocyte count of 11,974×106/L, with 95% neutrophils. Based on these findings, the patient was treated empirically for bacterial meningitis with intravenous (IV) vancomycin, ceftriaxone and dexamethasone pending culture results and sensitivities. Further blood work revealed a blood leukocyte count of 13.8×109/L, hemoglobin level of 123 g/L and platelet count of 154×109/L. His serum sodium level was 146 mmol/L, potassium level 3.6 mmol/L, chloride level 106 mmol/L, urea level 12.6 mmol/L and creatinine level 190 μmol/L.
Initial Gram stain of the CSF using the cytospin technique revealed abundant polymorphonuclear leukocytes and no organisms. Preliminary reports revealed growth of Gram-negative coccobacilli, and at this point metronidazole was also added to the treatment regime until an anaerobic cause was ruled out. Ultimately, the organism was identified from aerobic cultures as P multocida using the Vitek 2 identification system (bioMérieux, USA). Anaerobic cultures were negative. The isolate was sensitive to ceftriaxone, ampicillin and penicillin. Interestingly, a swab of the left ear performed on postoperative day 2 grew the same organism as that cultured from the CSF (growth on chocolate and blood agar; no growth on MacConkey’s or inhibitory mold agar). The empirical antibiotics and dexamethasone were discontinued, and the patient was started on a 14-day course of IV penicillin G at a dose of 2,000,000 units every 4 h. The patient made a rapid recovery from his meningitis, and he was discharged on postoperative day 10 to continue treatment as an outpatient. On further questioning, it was revealed that the patient was the primary caregiver of several pet cats and a dog, although he reported no history of bites. The pets were allowed on the furniture, including his bed, and would occasionally lick his face.


A 37-year-old female patient visited our clinic complaining of painless swelling on the ventral tongue that had been present for months. Intraoral examination revealed a 1 × 1 cm nodule on the left ventral tongue and an excisional biopsy was done. Light microscopy showed an infiltrative tumor composed mostly of clear cells with spindle cell stroma. The tumor cells were immunoreactive for cytokeratin AE1/3 (CKAE1/3) and p63, and the spindle cell stroma for smooth muscle actin (SMA). The original pathology report was myoepithelial carcinoma (MC) due to misinterpretation of the p63 immunoreactivity as myoepithelial differentiation. The diagnosis was soon revised to HCCC after recognizing this new entity.
The tumor comprised of cords and nests of tumor cells with skeletal muscle infiltration and perineural invasion (). The tumor cells had clear and pale eosinophilic cytoplasm (), fine nuclear chromatin with occasional small nucleoli, and no mitotic figure. The tumor cells were embedded in a characteristic dual hyaline and fibrocellular stroma (). No ductal formation was seen. On immunohistochemical stains, the tumor cells were positive for CKAE1/3 and p63 (), but negative for myoepithelial cell markers, such as SMA, CD10, S100, myosin, calponin, glial fibrillary acidic protein (GFAP), muscle specific actin (MSA), and desmin. The spindle cells in the fibrocellular stroma were positive for SMA and CD10 () but negative for CKAE1/3, p63, myosin, calponin, GFAP, MSA, S100, and desmin. The clear cells contained abundant glycogen highlighted by a diastase sensitive periodic acid-Schiff positive reaction (, ). The result of the mucicarmine stain for mucin was negative. Fluorescence in situ hybridization for EWSR1 showed a break-apart signal pattern in 25% of the tumor cells (), confirming the presence of EWSR1 gene rearrangement. Since the margin was focally involved by the tumor, the patient received further excision with safe resection margin, which showed no residual tumor.


In a fall on a large rake, this 76-year-old man was stabbed in the left neck by three tines []. He was taken to our emergency department with three metal tines penetrating his left neck; he was fully conscious and exhibited no neurological deficits. His vital signs were stable, and there was no apparent active bleeding. All laboratory data were normal.
A plain computed tomography (CT) scan showed that one of the tines penetrated between the C3/4 transverse foramen suggesting left vertebral artery damage. The other tine was attached to the hyoid bone and thyroid cartilage. His epiglottis was slightly swollen; there was no air leak, and he reported no respiratory discomfort. Other important structures such as the spinal cord and other major arteries and veins were intact. CT angiography (CTA) demonstrated obstruction of the left vertebral artery at the V2 segment proximal to the penetrating tine []. Because the metal artifact limited detailed evaluation we performed digital subtraction angiography (DSA) under general anesthesia to study his hemodynamic status and to select treatment. Angiography through the left vertebral artery revealed obstruction of the left vertebral artery and some oozing beside the metal tine []. Blood flow from the contralateral vertebral artery perfused the posterior fossa, and bilateral posterior communicating arteries were fetal type. We selected endovascular embolization to prepare for extravasation from the damaged vertebral artery.
After inserting a 6-Fr guiding catheter (Slim guide®, Medikit, Japan) into the left vertebral artery, we navigated two microcatheters (Excelsior®, Stryker Neurovascular, USA) into the proximal and distal sides to the injury point. It might be better that we use a stent to suppress the bleeding point and preserve anterograde blood flow, but vertebral artery at the injury point was too narrow to pass the stent device. A 4-Fr catheter was then inserted into the right vertebral artery for evaluation of the contralateral circulation. In the middle of slowly and carefully withdrawing the metal tine, we noted extravasation and decided that endovascular embolization was indicated. We first performed coil embolization at the entry - and the proximal side using the microcatheter inserted at the proximal side. Then we embolized the distal side to the site of entry using the distal microcatheter. Extravasation ceased after complete embolization of the left vertebral artery []. The tine was withdrawn, and the other two tines were removed at the same time. There was neither bleeding nor cerebrospinal fluid leakage after their removal and drainage tubes were inserted into the three stab wounds. As postembolization laryngoscopy revealed laryngeal wall damage by one of the tines, we performed tracheotomy.
Postoperative magnetic resonance imaging showed no evidence of cerebral infarction and single photon emission CT (SPECT) demonstrated normal cerebral perfusion []. His laryngeal swelling disappeared after a few weeks, and he was extubated. The perioperative administration of antibiotics prevented focal or general infection. He manifested no neurological deficits and was able to leave the hospital on foot.


A 9-year-old girl had asymptomatic lesions with a pink raised nodule and a papule around
the vulva and anus for 2 months ().
Physical examination revealed a firm, tender, 0.5 X 1.1 cm nodule on the left of the
vulva and a pink 0.4 X 0.3 cm papule around the anus. The dermoscopy using JD801G
(Jiangsu JEDA Science and Technology Development Co., Ltd, China) showed smooth, reddish
and piny surfaces of the lesions and a depression in the margin of the pink
papule, on which a crown vessel was observed ( and ). No similar lesions were
found anywhere else on her body. A detailed inquiry revealed no evidence of child abuse
and her family informed she often went swimming in a public pool. An excisional biopsy
was performed on both lesions. Histopathologic findings revealed a bulk of eosinophilic
and basophilic molluscum bodies surrounded by numerous keratinocytes ( and ). The transmission electron microscope showed numerous mature viral
particles (). Based on the
histopathological and electron microscopy findings, the diagnosis of molluscum
contagiosum was confirmed. There was no recurrence after a month of follow-up ().


A 15-month-old male child was admitted with the chief complains of high-grade fever for 7 days followed by altered sensorium for 1 day. On examination, child was febrile and unconscious. The child had uneventful perinatal history, no developmental delay, and no history of seizure. Blood pressure was 80/60 mmHg. Respiratory and heart rate were within normal limits. He had severe pallor. On systemic examination, he had hepatosplenomegaly with liver being around 4 cm palpable. Spleen was enlarged and measured to be 3 cm. All other systems were examined to be normal. Blantyre scoring showed 4/10. Blood investigations showed Hb was 5.8 mg/dl; total leucocyte count 12,400/cumm, and total platelet count 65,000/cumm. Other blood investigations were within normal limits. MPICT (antigen test) was positive and peripheral smear showed multiple rings of P. falciparum. Final diagnosis of cerebral malaria was made. Patient was treated using intravenous (IV) artesunate followed by ACT according to WHO guidelines. On follow-up fever decreased and sensorium improved, but on day 4 of the treatment child developed bilateral nystagmus with rapid component towards the right side. Child had no gait abnormality. Cerebrospinal fluid study and contrast-enhanced computed tomography were normal. After ruling out all other causes of nystagmus, it was diagnosed as an early complication of cerebellar involvement due to severe malaria. The child was followed-up and found nystagmus relieved with MPICT negative.


A 36-year-old female presented with a 6-month history of blisters on the dorsal aspects of her hands bilaterally with associated fragility and photosensitivity. This was also associated with a 2-year history of increased hair growth on her cheeks. She had been previously treated with oral antibiotics and topical steroid with no obvious improvement.
The patient had a medical background of asthma for which she was using twice daily fluticasone/salmeterol inhalations and salbutamol inhalations as required. Her only other medication was the oral contraceptive pill, which she had been taking long-term. She had no family history of PCT or haemochromatosis. The patient consumed approximately 15 alcoholic standard drinks per week.
On examination, the patient had multiple blisters and erosions distributed over the dorsa of her hands with a number of milia (fig. ). A punch biopsy of the right hand index finger and a perilesional biopsy of the left hand were performed. Histopathology showed a partly regenerated subepidermal blister with a heavy inflammatory cell infiltrate between the old roof and the regenerating base (fig. ). A small amount of festooning was evident. Immunofluorescence was strongly positive for IgG present along the basement membrane. The pattern was recognized as the ‘U-serrated pattern’, typical of antibodies to type VII collagen. Based on these findings, EBA was diagnosed and salt split skin preparation was not performed.
Although the initial attending doctor was dissuaded from a diagnosis of PCT on the basis of histology findings, subsequent review favoured PCT clinically and porphyrin studies later confirmed this (table ). Further blood results demonstrated normal full blood count, renal function and electrolytes. The patient had deranged liver function tests, but her liver screen did not demonstrate any abnormalities, including negative hepatitis B/C and HIV serologies and normal iron studies and alpha fetoprotein levels. A liver ultrasound demonstrated findings of fatty liver. Desmoglein 1 and 3 reactivity and subsequent collagen VII reactivity testing were all negative, excluding a concomitant diagnosis of pemphigus or EBA.
The patient was commenced on hydroxychloroquine 200 mg twice weekly and was given advice on mechanical and photoprotection. She was also commenced on regular venesections, aiming for a ferritin level of 50 μg/l to improve PCT symptoms.


A 50-year-old female presented for a second opinion after a 3-year history of spontaneous blistering affecting the extensor surfaces of her hands and forearms with associated scarring. She had been diagnosed with EBA by a previous clinician based on biopsy histopathology findings 3 months earlier. There was a cell-poor subepidermal vesicle containing only a few lymphocytes and with festooning of the dermal papillae and very little dermal inflammatory infiltrate seen (fig. ). Direct immunofluorescence studies revealed positive staining for IgG in a linear pattern along the dermoepidermal junction. All other markers were negative and salt split skin studies could not be performed due to technical difficulties.
The patient was initially treated with potent topical corticosteroids as well as low-dose oral corticosteroids and was still experiencing flares. She was then tried on 100 mg of oral doxycycline, which resulted in some improvement. Her past medical history included monoclonal gammopathy of uncertain significance which was managed conservatively. She also reported a positive family history for blistering of the hands affecting her maternal grandmother but no known family history of liver disease. She was a non-smoker and consumed 10–15 alcoholic standard drinks per week. She had been taking the oral contraceptive pill for many years but had recently ceased taking this.
On examination, the dorsal aspects of both hands were erythematous, with evidence of milia and post-inflammatory hyperpigmentation (fig. ). There was also associated hyperkeratotic scaling and lichenification of the skin. Based on this clinical appearance, further testing was performed, including full blood count, renal function, electrolytes and liver function, which were all normal. In addition no HFE mutations were detected and hepatitis B/C and HIV serologies were negative. Further urine and stool porphyrin studies showed elevated urine porphyrin, faecal porphyrin and porphyria/creatinine ratio, which confirmed the diagnosis of PCT (table ). Desmoglein 1 and 3 reactivity and collagen VII reactivity testing were negative, excluding a concomitant diagnosis of pemphigus or EBA. The patient was then commenced on hydroxychloroquine and doxycycline was ceased. She was given advice regarding photoprotection and decrease of her alcohol consumption. She was also advised to use gloves and zinc oxide sunscreen to provide physical protection.


A 55-year-old man underwent right above-elbow amputation due to synovial sarcoma. After the amputation, he suffered short-lasting stump pain. He underwent forequarter amputation of his right upper limb for recurrent sarcoma after 4 months. Two weeks later, he began complaining of phantom pain in the amputated limb, which he described as feeling ‘ice-cold’ and ‘cramping’. Six months later, he developed severe continuous pain in the lower back. Magnetic resonance imaging and bone scan showed multiple metastases in L3–5 and S1 vertebrae. He was prescribed oral tramadol- paracetamol combination, to which he initially responded, but after a month the pain became excruciating.
In the pain clinic, he complained of severe pain in the back and legs, which he rated as 9 of 10 on the verbal categorical pain score.[] He also reported burning, cramping intermittent PLP and rated it as 7/10. He said that ‘I know that I am dying of cancer. But please treat the pain, but I do not want any injections.’
His medical treatment of pain consisted of oral opioids 4 hourly followed by paracetamol, sustained-release morphine, sustained-release amitriptyline 50 mg and sustained-release pregabalin 150 mg twice daily, along with immediate-release morphine for his breakthrough pain. After 2 weeks of treatment, his pain in the back decreased to 2/10. However, he still rated his PLP as 7/10. The patient refused any invasive procedure as he was extremely anxious and had undergone multiple surgeries. After explaining the pros and cons and obtaining verbal consent from the patient and his wife, he was started on oral ketamine in liquid form (mixed with cola drink to mask its bitter taste) 50 mg 3 times a day. After 2 days, he reported pain relief. Over a period of 2 weeks, his phantom pain reduced to 3/10 without any side effects.
He was also continued on other medications along with 270 mg of oral sustained release morphine. Ketamine was stopped after about 4 months, considering its off-label use. However, 2 weeks later his phantom pain recurred, and oral ketamine was restarted. His general condition deteriorated further, and 2 weeks later he died at home while free from PLP on oral ketamine.


A 75-year-old man presented with progressive left eyelid swelling that started four weeks previously. His medical history included proximal gastrectomy because of a moderately differentiated gastric adenocarcinoma five years previously. The tumor cells expressed cytoplasmic cytokeratin- 7 (CK7) but not cytokeratin-20 (CK20). The eyelid swelling improved with prednisone administration. However, leg weakness and swelling developed one week prior, and the patient was consequently transferred to the oncology department of our hospital with a suspected spinal or vertebral metastasis. Orbital magnetic resonance imaging (MRI) revealed exophthalmos and a T2-weighted high-signal-intensity lesion accompanying fatty stranding on the preseptal and septal areas. The lesion was mostly enhanced and spread to the extraconal area; the inferior rectus muscle was also thickened without any definite mass. There was no specific finding showing an acute infarct on brain MRI. The overall imaging findings were suggestive of preseptal and septal cellulitis. Therefore, teicoplanin and ceftriaxone were administered because of concern about the potential for clinical complications of orbital cellulitis including visual disturbances and central nervous system manifestations.
Upon physical examination at our clinic, the patient presented with localized relatively ill-demarcated erythematous swelling and indurations at the left upper and lower eyelid (). Histopathological examination revealed diffuse infiltration with tumor cells that formed strands or clusters between the collagen bundles throughout the upper dermis (). The infiltrating tumor cells were round and polygonal and of variable size. Cytoplasm was eosinophilic, while the nuclei of the tumor cells were pleomorphic with conspicuous nucleoli with mitotic figures. Signet ring cells with intracytoplasmic mucinous vacuoles that exhibited nuclei laterally were also observed. Immunohistochemical staining showed the tumor cells expressed cytoplasmic epithelial membrane antigen (EMA) () and CK7 () but neither CK20 nor carcinoembryonic antigen (CEA). Therefore, a diagnosis of cutaneous metastasis of poorly differentiated gastric adenocarcinoma with signet ring cells was made. The patient's condition deteriorated rapidly, and he died two weeks later.


A 66-year-old woman was referred to us by the gynecology clinic because of her progressively aggravated pitting edema of the bilateral lower extremities; in addition, she suffered from intermittent abdominal pain, persistent anemia with intermittent hematuria and hematochezia, and all of these symptoms had occurred during the previous 4 years.
26 years ago, she received a radical hysterectomy with pelvic lymphadenectomy and external irradiation at a dose of 6100 cGy for the treatment of her uterine cervical carcinoma (stage IB). About 23 years ago, she began to suffer from intermittent hematochezia and hematuria. She had received intermittent transfusions of packed RBCs, about a total of 8 to 10 pints yearly, under the presumptive diagnosis of radiation colitis and cystitis. 20 years ago, she experienced an episode of liver abscess that was treated by liver surgery. 7 years ago, gross hematuria had developed and she was diagnosed as having bladder cancer (stage I) with a background of radiation cystitis, for which she had undergone a transurethral bladder resection.
When we examined the patient, we observed severe anemia and a grade 3 pitting edema on the affected parts of her body, and cachexia was also noticed. Her abdomen was soft and mildly distended without any shifting dullness. Bowel sounds was moderately increased and there was neither tenderness nor rebound tenderness. The liver was not palpable and a previous operation wound scar was found on the right upper abdomen.
The laboratory tests revealed microcytic, hypochromic anemia (hemoglobin: 6.7 g/dL, normal: 12.0–18.0 g/dL) with a serum iron level of 17 g/dL (normal: 37–170 g/dL), a total iron binding capacity of 123 g/dL (normal: 250–450 g/dL), and a ferritin level of 78 ng/mL (normal: 20.0–300.0 ng/mL). The levels of vitamin B12 and folate in the serum were normal and the stool occult blood test was positive. The anemia was due to her chronic blood loss and chronic disease. The serum protein level was 4.7 g/dL (normal: 6.3–8.2 g/dL), the albumin level was 1.6 g/dL (normal: 3.5–5.1 g/dL), the cholesterol was 87 mg/dL (normal: 100–200 mg/dL), and the serum total bilirubin, AST/ALT, alkaline phosphatase and prothrombin time were all normal. The BUN and creatinine levels were normal. The urinalysis results revealed microscopic hematuria (RBC 5–9/HPF) and pyuria (>30/HPF) without proteinuria. Urine cytology didn’t reveal any malignant cells, and the urine culture was negative. Stool examination was negative for fat and parasites. The result of the alpha-1- antitrypsin (α1-AT) clearance test using 24hr stool collection was 169 mg/24hr (normal: 0.0–27.0 mg/24hr), which was markedly increased and diagnostic for a protein-losing enteropathy. The small bowel series showed multifocal thumb-printing lesions in the ileum with delayed passage (). On the barium enema, there was diffuse luminal narrowing with the loss of haustral markings in the rectum and sigmoid colon. (). The sigmoidoscope findings were diffuse mucosal friability and vascular ectasia, which were gross and microscopic findings compatible with radiation colitis (). Chest X-ray, electrocardiogram, echocardiogram, lung function tests and upper endoscopic findings were unremarkable. Abdominal CT scan showed liver cysts (size: 2×2 cm and 0.8×0.8 cm) in segments 4 and 6, respectively. Cystoscopic examination showed the mucosal thickening with edema and vascular ectasia, but there was no evidence of tumor recurrence. 99mTc-human serum albumin scintigraphy showed localized radioactivity in upper abdomen () on the 24hr delay image, which meant there was a small amount of bowel leakage of radiolabeled albumin in the ascending colon. 99mTc-labeled dextran lymphoscintigraphy showed a decreased inguinal uptake in the left ileac lymph nodes with delayed migration and collateral circulations (), suggesting lymphatic obstruction in that area.
The patient’s symptoms persisted and she required transfusion of 1 pints of packed RBCs due to the anemia, and she also received infusion of 25 grams of albumin in a month’s time to reduce her edema and dyspnea.
About 5 months later, she was re-admitted with symptoms of fever and severe dyspnea. The laboratory findings showed azotemia (BUN 89 mg/dL, serum creatinine 5.0 mg/dL), leukocytosis (14,500/mm3, neutrophils 89%), hematuria and pyuria. On urine culture, Escherichia coli and Morganella morganii were evident. Her hemoglobin level was 9.6 g/dL, and the serum protein (4.8 g/dL) and albumin (1.6 g/dL) levels were very low. Abdominal ultrasonography showed bilateral hydronephrosis. The patient’s condition was severe; she had a urinary tract infection associated with urinary flow stasis that was probably due to a neurogenic cause because there was no mechanical cause for the obstruction on the cystoscopic examination. The patient received prompt administration of broad spectrum antibiotics and percutaneous nephrostomy on the right kidney. The patient condition then gradually improved with recovery of the serum creatinine to 1.9 mg/dL. However, a generalized seizure suddenly developed on the 18th day of admission. The magnetic resonance image (MRI) of the brain demonstrated diffuse brain atrophy and multiple small high signal intensity lesions in the bilateral hemispheres, and this was suspected of being small vessel disease or vasculitis. Fluorescent antinuclear antibody and anti-neutrophil cytoplasmic antibody testing were both negative. The level of complement 3, complement 4, fibrinogen and fibrinogen degradation product were within the normal limits. The seizure was not controlled with phenytoin and carbamazepin, and it became progressively aggravated in terms of frequency and duration. On the 32nd day of admission, she finally expired after a severe attack of seizure.


A 30-year-old male patient was admitted to the Department of Internal Medicine at our hospital for the evaluation and treatment of ptosis of the right eye and blurred vision for 1 month. The patient also experienced fatigue and generalized weakness. He was neither a smoker nor an alcoholic, and had no history of drug abuse, including herbs. His family history was unremarkable. On physical examination, his height was 173 cm, body weight was 63 kg. Body temperature was 36.5°C, pulse rate was 20/min, respiratory rate was 20/min and blood pressure was 120/70 mmHg. He had a chronically ill appearance and the sclera was not icteric. On respiratory and cardiac auscultation, no abnormal sound heard. On abdominal examination, abnormal tenderness and hepatosplenomegaly were not detected. On neurologic examination, the function of the cranial nerves, including pupillary reflexes of both eyes, was intact and the pathologic reflexes, including the Babinski sign, were not detected. A pathognomonic sign of myasthenia gravis, tensilon test, was positive. Hematologic tests showed WBC 3900/mm3, hemoglobin 13.4 g/dL, platelet 139,000/mm3, and blood chemistry showed total bilirubin 1.4 mg/dL, alkaline phosphatase 82 U/L, total protein/albumin 7.3/5.0 g/dL, gamma GT 32 U/L, BUN/creatinine 10/0.9 mg/dL, LDH 371 U/L, CK 57 U/L, Na/K/Cl 145/4.1/106 mmole/L. However, liver enzymes were markedly elevated (AST/ALT 400/777 U/L) and prothrombine time was slightly prolongated (INR 1.35). Anti-acetylcholine receptor antibody titer was elevated to 3.8 nmole/L (normal < 0.2 nmole/L). Tests of etiologic agents for viral hepatitis were anti-HAV IgM (−), HbsAg (−), anti-HBs (−), HBV-DNA (−), anti-HCV (−), HCV-RNA by PCR method (−), anti-HDV by RIA method (−), HEV (−), anit-CMV IgM (−), and anti-EBV (−). Autoantibody were ANA (+, 1:40, speckled pattern), anti-smooth muscle Ab (−), anti-LKM Ab (−), anti-microsomal Ab (−), anti-mitochodrial Ab (−), anti-dsDNA Ab (−), anti-smooth Ab (−), anti-thyroglobulin Ab (−). Screening tests for Wilson’s disease showed normal values, serum copper 99 g/dL and serum ceruloplasmin 20 mg/dL, and Kayser-Fleisher rings were not observed. Alpha1-antitrypsin was 244 mg/dL and serum choline esterase was 1605 IU/L. These were within normal value. Thyroid fuction tests showed T3 153 ng/dL, T4 11.1 g/dL, TSH 2.33 IU/mL. The urinalysis was normal. A chest X-ray showed no active parenchymal lung lesion or mass, but the chest CT scan showed 3×4×5 cm-sized low-attenuated homogenous oval shaped mass with sharp margin at the anterior mediastinum. There was no enlargement of lymph node in thoracic cavity. The mass was diagnosed as thymoma, radiologically (). Abdominal sonography showed no abnormal findings. His HLA typing was A11, A31, B51, B13, Cw4, Cw6, DR09 and DR12. In the follow-up liver function tests, AST/ALT were elevated to 331/918 and liver biopsy was performed. Biopsy showed distorted lobular architecture and widening of portal tracts by chronic inflammatory cell infiltration with foci of piecemeal necrosis and fibrosis. Thus it was diagnosed as chronic active hepatitis (). According to the scoring system of International Autoimmune Hepatitis Group, his pretreatment score was 14, which is compatible with the probable diagnosis of autoimmune hepatitis. He was treated with prednisone 30 mg/day per oral and 20 days later AST/ALT decreased to 45/73 IU/L and, thereafter, he received thymectomy. On operative fields, solid mass (3×4×5 cm) was identified at the anterior mediastinum. It was encapsulated with yellow gelatin-like material. Histology confirmed that the thymoma was encapsulated, non-invasive and mixed type (). After thymectomy, ptosis and blurred vision disappeared. Prednisone was gradually tapered and stopped, but AST/ALT level was elevated up to 239/393 U/L. Therefore, he was treated again with prednisone, 10 mg/day, and liver function returned to normal value. His post-treatment score was 17, which is compatible with the definite diagnosis of autoimmune hepatitis.


A 56-year-old woman was referred to our hospital with complaints of chronic diarrhea, weight loss, indigestion and abdominal pain lasting for 11 months. Before admission, the patient visited a private clinic and underwent gastroscopic and colonoscopic examinations. Gastroscopic examination showed small gastric ulcerations on the antrum and colonoscopic examination showed multiple punched-out ulcerations and erosions on the entire colon, including the sigmoid colon to the terminal ileum. Biopsy results were not diagnostic at that time. Under the clinical impression of intestinal tuberculosis, she received 2 months of antituberculosis medication without improvement of symptoms. The follow-up biopsy result of colonoscopic examination showed intestinal lymphoma of T-cell type. The patient was then referred for further evaluation. On admission, the patient looked pale, emaciated and dehydrated. An eleven kg weight loss was noted during the last 6 months (47kg--> 36kg). She denied fever or night sweating except complained of profuse watery diarrhea (5–6/day) and indigestion. She denied aggravation of diarrhea symptoms after wheat ingestion. No improvement of diarrhea was found by withdrawal of wheat intake. There was no palpable lymphadenopathy or hepatosplenomegaly. Diffuse abdominal tenderness on the whole abdomen was noted on abdominal physical examination. Initial laboratory findings were as follows: WBC 8,300/mm3, Hb 10.7 g/dL, Hct 32.6%, platelet 409,000/mm3, urine analysis was normal, stool occult blood was negative, serum total protein 5.2 mg/dL, albumin 2.3 mg/dL, serum LDH 128 IU, calcium 7.9 mg/dL, phosphorous 4.4 mg/dL, ALT/AST 12/13 U, cholesterol 101 mg/dL, BUN 13 mg/dL and creatinine 1.0 mg/dL. Chest X-ray was normal. Gastroscopic examination showed diffuse irregular nodular and depressed lesions on the angle and the lower body and multiple red spots on the antrum, suggesting erosive gastritis. But esophagus and stomach biopsies showed diffuse infiltration of lymphoma cells with epitheliotropic features (, ). Duodenal biopsy also revealed lymphoma cell infiltration, and villous atrophy and blunting were found. Colonoscopic findings were the same as noted previously () and the biopsy showed diffuse infiltration of small to medium-sized lymphoma cells (). The infiltrated lymphoma cells were immunostained for UCHL-1 (+), CD 3 (+), MT-1 (+), L-26 (−) and MB-2 (−) (Figure 1.2.4). Small bowel series also showed chronic granulomatous lesions on the cecum and the distal ileum. Abdominal CT scan revealed diffuse wall thickening of the bowels, including the esophagus, the stomach and parts of the small bowel and colonic loop. There was no evidence of involvement of lymphoma on bilateral bone marrow biopsies. Finally, the patient was diagnosed as primary intestinal T-cell lymphoma involving the entire gastrointestinal tract (Ann Arbor Stage IVB) with malabsorption. She was treated with combination chemotherapy including cyclophosphamide, adriamycin, vincristine and prednisone (CHOP). Although after two cycles of chemotherapy the initially observed bowel wall thickening disappeared on follow-up abdominal CT scan and no definitive nodular or ulcerative lesions were noted, on follow-up gastro-and colonoscopic examinations lymphoma cells remained on the biopsy. Despite further treatment with 4 cycles of systemic chemotherapy, only a partial response was achieved.
The patient was readmitted to our hospital with complaints of hematochezia. Colonoscopic examination showed internal hemorrhoid and diffuse hyperemic mucosa and multiple ulcerations were noted throughout the rectum to cecal area, suggesting relapse of lymphoma. After two cycles of salvage chemotherapy with etoposide, dexamethasone, adriamycin and cisplatin (EDAP), the bowel lesions were slightly improved but lymphoma cells were still found, indicating refractory to chemotherapeutic agents, agten then, the patient was treated without a specific treatment except supportive care. Eventually, the patient died of spontaneous bowel perforation.


A 23-year-old Korean man was admitted with a chief complaint of weakness and decreased sensation of left arm and leg for 7 days. He had suffered from polyarthralgia and generalized edema for 2 years, and took herbal medicine occasionally. He had a history of hair loss, but he didn’t complain about allergies, photosensitivity, skin rash, oral ulcer, dry mouth, dry eye and Raynaud’s phenomenon. On examination, he was normotensive (110/70 mmHg) but febrile (37.8 C). He appeared acutely ill, but showed alert mentality. There were no pathologic lesions in his ears, eyes, nasal or oral mucosa. The pulmonary and cardiac examinations were normal. The abdominal examination revealed shifting dullness. His joints were unremarkable, there was no cutaneous vasculitis and all peripheral pulses were present with no arterial bruits. Neurologically, he had left-side paresthesia, hemiparesis and a brisk, deep tendon reflex, but other abnormal neurologic signs were not noted.
On admission, white blood cell count was 6.4×103/mm3 with left shift (seg.neut. 55%, band neut. 32%, lymph. 10%, mono. 3%). Hematocrit was 15%, hemoglobin 5.5gm/dl, and the reticulocyte count was 3.5%. The erythrocyte sedimentation rate was elevated at 23 mm/h (Westergren). The direct Coomb’s test was positive. Review of the peripheral blood smear showed unremarkable red cell morphology. Urinalysis showed 100 mg/dl protein, 5–7 white cells and many red cell. The urine hemosiderin and hemoglobin were negative. Renal function evaluation revealed blood urea nitrogen 26 mg/dl, creatinine 1.5 mg/dl, 24 hr urine protein 3.4 gm/day, and the creatinine clearance was 55 ml/dl. The serum electrolyte were normal, except for potassium of 3.2 mmol/I. The AST was 34 IU/I, ALT 42 IU/I, alkaline phosphatase 48 IU/I, total bilirubin 0.7 mg/dl, total protein 5.8 gm/dl and albumin 2.7gm/dl. The platelets were 22×103/ml, PT 10.7/100 (sec/%) and aPTT 34/28(sec, patient/control). Rheumatoid factor was negative and ANA was positive (speckled pattern, titer 1:160). The anti-dsDNA antibody was above 100 U/ml (N; 0–25 U/ml). The anti-Sm, anti-RNP, anti-Ro and anti-La antibodies were all positive. Complement levels were decreased at C3 26.5 mg/dl (N; 52.6∼120 mg/dl), C4 8.0 mg/dl (N; 20.5∼49 mg/dl). Anticardiolipin antibodies (aCL) were positive for both IgG and IgM (by ELISA) and VDRL was reactive, TPHA nonreactive and FTA-Abs negative. The levels of protein C, protein S and antithrombin III were within normal limits. Plain radiology of chest was normal. Abdominal ultrasonography revealed moderate amount of ascites, splenomegaly and diffusely increased renal parenchymal echogenicity. Magnetic resonance imaging (MRI) brain scan revealed ischemia in right parieto-temporal white matter (). EKG showed sinus bradycardia and low voltage on limb leads. Echocardiography confirmed mild pericardial effusion without vegetation and thrombus.
Course: He was diagnosed as APS with cerebral thrombosis in SLE. He was given methylprednisolone pulse therapy and oral prednisolone. Kidney biospy showed mixed class III and V(focal and segmental proliferative glomerulonephritis and membranous lupus glomerulonephritis) lesion with activity score 4/24, and chronicity score 1/21. After the kidney biopsy, anticoagulation therapy was begun with warfarin.


A 27 year old pregnant woman was admitted to the hospital with severe dyspnea. She had been free of cardiac symptoms until one month prior to admission (25 weeks gestation). She began to be treated with digoxin and diuretic therapy. On two days before admission, she suffered from severe dyspnea and orthopnea.
Physical examination showed an acutely distressed woman in moderate respiratory distress. The blood pressure was 100/60 mmHg and the pulse rate was 120/min; respirations were 28/min. The jugular venous pressure was increased to 12 cm above the sternal angle. Cardiac examination revealed a prominent right ventricular heaving. The first heart sound was increased and the second sound was narrowly split with an accentuated pulmonic component. There was a grade 4/6 diastolic rumble with presystolic accentuation at the apex. Abdomen was protruded due to enlarged uterus. Height of uterine fundus was up to the umbilicus. There were no abnormal findings on gynecological examination except enlarged uterus due to 26 weeks gestation. The obstetrical ultrasonographic examination revealed no abnormal findings on the fetus. There was no peripheral edema.
The electrocardiogram showed the findings of left atrial enlargement. Two dimensional and Doppler echocardiographic findings were consistent with severe mitral stenosis. There was evidence of left atrial enlargement, normal left ventricular dimension and function. There was no evidence of mitral regurgitation and echogenic mass in left atrium.
After intensive treatment with diuretics she could remain in the supine position. To protect the fetus from radiation, she was wrapped in a lead shield from the diaphragm to the symphysis pubis. Fetal monitoring, including fetal heart rate, was done during the procedure.
Using the percutaneous femoral approach, a 7 Fr. Swan-Ganz catheter was placed in the pulmonary artery and an 8 Fr. pigtail catheter was placed in the left ventricle. The pressures, thermodilutional cardiac output and oximetry were recorded. After prevalvuloplasty measurements (), transseptal catheterization was accomplished from the right femoral vein using a modified Brockenbrough needle and an 8 Fr. Mullins transseptal sheath (USCI). Systemic anticoagulation was achieved with 5000 units of heparin given after successful transseptal catheterization. Left heart pressures and transvalvular gradients were then measured directly to corroborate the previously obtained wedge pressure measurement. The 7 Fr. wedge ballon catheter was advanced through the mitral and aortic valves to the descending aorta, followed by insertion of a 0.035 inch, 260 cm long Teflon-coated exchange guide wire through the flow directed catheter. The sheath and balloon catheter were then removed, leaving only the guide wire in place. After dilating the septal puncture site with 8 mm balloon (Meditech Inc.), a second guide wire was introduced to the descending aorta through the double lumen catheter which was inserted through the first guide wire. After removal of this catheter, two 18 mm balloon catheters (9Fr.) were advanced across the septum through the left atrium and positioned across the mitral annulus. Two balloon inflations with a mixture of saline solution and iodinated contrast medium were then performed until the indentation in the balloon due to the mitral stenosis disappeared.
Immediately after catheter valvotomy, all hemodynamic measurements, including determinations of transvalvular gradient and cardiac output, were repeated (). shows the simultaneous left atrial and left ventricular pressure before and after balloon dilatation. there was a decrease in mean diastolic gradient across the mitral valve to 8 mmHg and an increase in cardiac output to 6.6 liters/min. The mitral valve area was increased to 2.4 cm2. Pulmonary arterial systolic pressure decreased to 46 mmHg. Right heart oximetry demonstrated no evidence of a left to right shunt across the atrial septum. To minimize radiation exposure to the fetus, left ventricular cineangiogram was not performed. However, definite evidence of mitral regurgitation was not detected by physical examination and by Doppler examination of the mitral valve. The procedure was completed in 2 hours (fluoroscope time; 35 min.) and the patient tolerated it well with minimal discomfort. There was no evidence of fetal distress and the amount of radiation was 0.13 rem during the procedure.
She delivered a 3.51 kg, healthy, full-term boy without any evidence of fetal abnormalities. Follow-up examination of this boy for 17 months has shown normal growth and development.


A 27-year-old man was admitted to Yong Dong Severance Hospital, Yonsei University Medical Center, for evaluation and management of mediastinal mass.
Three years prior to admission, he found mediastinal mass on computerized tomographic (CT) scan performed in another institution.
At admission, he was noted to be with generalized weakness and right chest pain, but he denied cough, sputum and dyspnea.
The temperature was 38.6°C, the pulse rate 72/min and the blood pressure 110/70 mmHg.
On examination, he appeared chronically ill, the skin was warm and dry, the head and neck were normal and cervical lymphadenopathy was not found, the lungs were clear and the heart was normal.
The abdomen and extremities were normal.
The urine was normal and the hemoglobin was 13.5gm/dl; hematocrit 41.9%; white cell count 5,500/mm3 with 64 percent neutrophils, 35 percent lymphocytes and 1 percent eosinophils, the serum electrolytes and liver function tests were normal. T3: 150 ng/dl, T4: 7.1 ug/dl, FT4: 1.6ng/dl and TSH was 1.5 uU/ml.
The electrocardiogram showed right axis deviation and tall R wave in right precordial leads, and the sputum exanination did not suggest malignancy or tuberculosis.
Chest roentgenogram () revealed well defined mediastinal mass on the right paratracheal area.
Chest CT scan revealed large multi-cystic mass lesion on the right paratracheal area which was attached to the lower pole of right thyroid gland, displaced the superior vena cava anteriorly and extended to the level of the azygous arch vein ().
The lung perfusion scan and whole body bone scan were normal and the thyroid scan revealed cold area in the lower pole of right thyroid gland.
The fiberoptic bronchoscopy revealed extrinsic mass effect without mucosal lesions on right intermediate bronchus.
The pulmonary function studies showed mild restriction with FEV1:2.85 L/sec and FVC: 3.49 L/sec.
He underwent operation under the impression of mediastinal mass.
8×8 cm sized mass composed of multi-cystic and solid part was located in the pericardium and excised easily from the surrounding structures and the feeding vessel maybe originated from the subclavian artery.
Grossly, the mass was composed of several cystic lesions and yellow solid lesion, resembling unexpanded lung tissues () and the surface of the mass shows a feeding vessel ().
Mlicroscopically, the cyst wall showed a bronchial tissue which was composed of ciliated columanar epithelium, submucosal gland and cartilage () and solid portion of the mass showed alveolar space filled with inflammatory cells such as macrophages or lymphocytes ()