Abstract
A 59-year-old female with von Recklinghausen's disease (VRD) was found to have elevated serum hepatobiliary enzymess. On abdominal CT scan, dilatation of the biliary tract and a tumor at the second portion of the duodenum were noted. On endoscopy, an irregularly shaped tumor was seen on the papilla of Vater ; based on the pathology of the biopsy specimen, the lesion was diagnosed as a carcinoid tumor. The patient had a pylorus-preserving pancreatoduodenectomy. In addition to the ampullary tumor, duodenal submucosal tumors were seen in the resected specimen ; based on the histopathology, a somatostatin-producing carcinoid tumor of the papilla of Vater and gastrointestinal stromal tumors of the duodenum were diagnosed. It has been reported that various gastrointestinal lesions are occasionally found in VRD patients. This paper reports the case and reviews the related literature.
