Somatostatin-containing carcinoid tumor of the duodenum in neurofibromatosis: report of a case.

We report a case of von-Recklinghausen's disease presenting with obstructive jaundice and found to have a somatostatin-containing carcinoid tumor in the papilla of Vater and a small neurofibroma in the duodenum. A 42-year-old woman with von-Recklinghausen's disease presented with intermittent jaundice, pruritus, and mild steatorrhea of a two-year duration. Abdominal ultrasonography and computed tomography showed dilated intrahepatic ducts, common bile duct and pancreatic ducts. Duodenoscopy showed a tumor at the papilla of Vater, but a preoperative biopsy failed to provide a definite diagnosis. Laparotomy revealed a yellowish tumor at the papilla of Vater and another nodule on the mesenteric side of the second section of the duodenum. Microscopically, the tumor at the papilla of Vater was found to be a somatostatin-containing carcinoid tumor. The small nodule on the mesenteric side was a neurofibroma. The jaundice, pruritus and steatorrhea disappeared after surgery.