A 26-year-old woman was admitted to our hospital with relapse of ulcerative colitis (UC). We diagnosed it as a refractory UC because intravenous corticosteroid therapy had no effect. Intravenous cyclosporine therapy and other conventional therapies did not lead to remission. Then the possibility of infliximab was discussed with the patient and her parents and treatment with infliximab was started. Infliximab was infused intravenously at a dose of 5mg per kilograms of body weight. Immediately after the first infusion, clinical symptoms improved, and clinical remission was achieved within 12 weeks. It is suggested that infliximab can be effective for refractory UC.
A 62-year-old Japanese woman with ulcerative colitis (UC) had episcleritis and erythema nodosum. Oral administration of corticosteroid with granulocytapheresis improved all these diseases. Extraintestinal manifestations such as ocular and skin complications are infrequent, especially in Japan, in patients with UC. Although concurrent onset of episcleritis and erythema nodosum associated with UC is also extremely rare, clinical course in this case suggests a possible association among ulcerative colitis, episcleritis and erythema nodosum.
Undifferentiated pleomorphic sarcoma was a well-known tumor that usually involves the soft tissues. However, in the alimentary tract, it was seldom reported. A 55-year-old man was admitted to our hospital with lower abdominal pain. Endoscopic examination showed a protruding tumor occupied the lumen of the descending colon. Because of the colon obstruction by the tumor, a left hemicolectomy with en bloc lymph node dissection was performed. Main occupied lesion of the tumor was mesenterium and submucosa, with no epithelial dysplasia. Also, dissemination of the tumor was recognized in the mesenterium and greater omentum. Based on histological and immunohistological studies, the tumor was diagnosed as undifferentiated pleomorphic sarcoma.
A 79-year-old man was admitted with hematemesis. Emergency upper gastrointestinal tract endoscopy revealed bleeding from the papilla of Vater. Computed tomography showed cholecystitis with a gallstone and a hematoma in the gallbladder. Angiography demonstrated a pseudoaneurysm of the anterior branch of the right hepatic artery. Emergency selective transcatheter arterial embolization (TAE) was performed, with intravascular microcoils, and complete occlusion of the pseudoaneurysm was achieved. This case shows embolization is effective in the treatment of pseudoaneurysm rupturing in the gallbladder.
A 61-year-old woman was referred to our hospital for a double balloon endoscopy (DBE) examination of small intestine. She had undergone laparotomy for a perforated ulcer of the 3rd portion in the duodenum 3 years prior to this admission. Esophagogastroduodenoscopy at the previous hospital revealed multiple ulcers in the 2nd and 3rd portions in the duodenum. DBE revealed multiple ulcer scars in the proximal jejunum. Zollinger-Ellison syndrome was suspected from the distribution of the ulcers and scars. Serum gastrin was high and a selective arterial calcium injection test showed a step up of gastrin level only in the gastroduodenal artery area. We diagnosed a gastrinoma located on the ventral side of the 2nd portion of the duodenum from imaging studies. The tumor was extirpated and histologically found to be a neuroendocrine tumor in a lymph node. Serum gastrin level decreased to the normal range a day after surgery.
We experienced a rare case of autoimmune pancreatitis with sclerosing cholangitis, retroperitoneal fibrosis and interstitial pneumonia. The patient was a 68-year old man and had abnormal liver function with jaundice. We performed endoscopic retrograde cholangiopancreatography (ERCP) and needle biopsy of the liver and pancreas. We obtained histological findings such as dense infiltration of plasma cells and lymphocytes and remarkable fibrosis and diagnosed AIP. The patient received treatment by prednisolone and thereafter developed bladder cancer and gastric cancer. He has been followed up for 4.5 years with no recurrence.
A 58-year-old man with a complaint of sever epigastralgia was referred to our hospital. At a previous hospital, he had been initially diagnosis as having ruputured aneurysm of the head of the pancreas. A final diagnosis of arteriovenous malformation of the head of the pancreas by MDCT and abdominal angiogram, and underwent pancreatoduodenectomy. Major feeding arteries were the gastroduodenal artery and inferior pancreatoduodenal artery, and the major drainage vein was the gastrocolic trunk. This case was also diagnosed as Osler-Weber-Rendu disease because of the presense of three of the four criteria for this disease: habitual epistaxis, oral telangiectasia, and angiodysplastic lesion in the internal organ. This is the first reported case of arteriovenous malformation with Osler-Weber-Rendu disease in Japan.
Von Hippel-Lindau disease (VHL disease) is an inherited neoplasia syndrome. VHL disease which frequently complicates pancreatic lesions is rarely diagnosed by existence of pancreatic involvements. We report two cases of VHL disease with pancreatic lesions. The first patient was a 40-year-old woman. Adrenal pheochromocytoma, spinal hemangioblastoma and pancreatic endocrine tumor were resected. The second case was a 68-year-old woman with past surgical histories included cerebellar and spinal hemangioblastoma. Subtotal pancreatectomy was performed for multiple serous cystadenoma. IPMN which has been never reported as pancreatic involvement of VHL disease were documented by imaging diagnosis in the first case, and by histological examination in the second case. We considered VHL disease from coexistent multiple tumors include pancreatic involvements and finally diagnosed by genetic examination in both cases. Care should be taken regarding the patient's right for treatment against for the genetic disease. We hold a genetic conference composed of multidisciplinary team. Consequently we detected another VHL disease patient from patient's family.