Familial occurrence of congenital bile duct dilatation.

Congenital bile duct dilatation (CBD) that developed in a parent and son is presented. Familial occurrence of CBD is rare, with only a few male cases having been reported. Since the initial report of CBD occurring in siblings in 1981, a total of 20 cases (10 pairs) have been published as of 2007. Clinical and genetic features of CBD are discussed.

Carcinoembryonic antigen-producing adrenal adenoma resected using combined lateral and anterior transperitoneal laparoscopic surgery.

A 74-year-old woman presented with symptoms consistent with hyperadrenocorticism and hyperca-techolaminism. She had a cushingoid appearance and her cortisol level was elevated. Her serum dopamine and noradrenalin levels were also elevated. Computed tomography detected a left adrenal mass measuring 3.5 cm multiply 3.0 cm in diameter. Metaiodobenzylguanidine scintigraphy was negative. Unexpectedly, the serum Serum carcinoembryonic antigen (CEA) level was elevated. Fluorodeoxyglucose positron emission tomography showed increased uptake in the adrenal tumor only, with a maximum standardized uptake value of 2.8. Selective venography and blood sampling revealed that the concentrations of cortisol, catecholamines and CEA were significantly elevated in the vein draining the tumor. A diagnosis of CEA-producing benign adenoma was made. After preoperative management, we performed a combined lateral and anterior transperitoneal laparoscopic adrenectomy. Her vital signs remained stable during surgery. Histopathological examination revealed a benign adenoma. Her cortisol, catecholamine and CEA levels normalized immediately after surgery. We present, to the best of our knowledge, the first case of CEA-producing adrenal adenoma, along with a review of the relevant literature, and discuss our laparoscopic surgery techniques.

Somatostatinoma of the papilla of Vater with multiple gastrointestinal stromal tumors in a patient with von Recklinghausen's disease.

We report a case of somatostatinoma of the papilla of Vater with multiple gastrointestinal stromal tumors in a patient with von Recklinghausen's disease (VRD). A 64-year-old woman who had had recurrent attacks of acute pancreatitis and cholangitis was found, on gastroduodenal endoscopy, to have a tumor of the papilla of Vater and multiple submucosal tumors of the stomach and duodenum. Numerous submucosal tumors were observed in the stomach, duodenum, and jejunum, and total excision of the papilla of Vater and resection of the duodenal and jejunal submucosal tumors was performed. The tumor of the papilla of Vater showed the histologic appearance of a dense proliferation of tumor cells in acinar form, from the duodenal mucosa to the muscle layer, and psammoma bodies were revealed within the tumor. Immunohistologically, the tumor cells were intensely positive for somatostatin. The submucosal tumors of the duodenum and jejunum were negative for smooth muscle actin, s-100, and neuron-specific enolase (NSE), and positive for CD34 and c-kit, and they were diagnosed as gastrointestinal stromal tumors (GISTs) according to the strict definition. The only 25 cases of papilla of Vater somatostatinoma associated with VRD to have been reported in the English-language literature since 1982 are reviewed, as well as our own case.

Inhibitory effect of loxiglumide (CR 1505), a cholecystokinin receptor antagonist, on N-nitrosobis(2-oxopropyl) amine-induced biliary carcinogenesis in Syrian hamsters.

We evaluated the cholecystokinin (CCK) receptor antagonist loxiglumide (CR1505) for a possible inhibitory effect on biliary carcinogenesis in a hamster model. Experimental group I underwent cholecystoduodenostomy and ligation of the distal end of the common bile duct, after which the animals were injected with N-nitrosobis(2-oxopropyl)amine (BOP) alone. Group II, after the same surgical procedure as in group I, were given injections of BOP and then given loxiglumide in their diet. The sham-operated group underwent simple laparotomy and then were given injections of BOP. Loxiglumide significantly inhibited BOP carcinogenicity in the gallbladder and extrahepatic bile duct but not in the intrahepatic bile ducts or pancreas. Autoradiography showed that loxiglumide significantly suppressed (125)I-Bolton-Hanter (BH)-CCK-8 binding to CCK receptors in the gallbladder and extrahepatic bile duct but not in the liver or pancreas, and CCK binding to its receptors was observed in an area identified as cancer tissue. CCK receptor antagonists have an inhibitory effect on BOP carcinogenesis in the extrahepatic biliary tract, including the gallbladder and extrahepatic bile duct, of Syrian hamsters. The difference in the inhibitory effect of loxiglumide on biliary carcinogenesis in hamsters according to site may be due to differences in CCK receptors or the affinity of loxiglumide for such biliary tract organs. A difference between carcinogenesis in the intrahepatic bile ducts and extrahepatic biliary tract may be another reason.