Case Report
Copyright ©2008 The WJG Press and Baishideng. All rights reserved.
World J Gastroenterol. Feb 28, 2008; 14(8): 1299-1301
Published online Feb 28, 2008. doi: 10.3748/wjg.14.1299
Carcinoid tumor of the common bile duct: A rare complication of von Hippel-Lindau syndrome
Otmane Nafidi, Bich N Nguyen, André Roy
Otmane Nafidi, Department of Surgery, Saint-Luc Hospital, CHUM, Montréal, Québec H2X 3J4, Canada
Bich N Nguyen, Department of Pathology, Saint-Luc Hospital, CHUM, Montréal, Québec H2X 3J4, Canada
André Roy, Department of Surgery, Saint-Luc Hospital, CHUM, 1058 Saint-Denis Street, Montréal, Québec H2X 3J4, Canada
Author contributions: Nafidi O wrote the paper and was involved in the care of the patient; Nguyen BN was the pathologist who studied the specimen; Roy A was the attending surgeon who performed the surgery.
Correspondence to: Dr. André Roy, Department of Surgery, Saint-Luc Hospital, CHUM, 1058 Saint-Denis Street, Montréal, Québec H2X 3J4, Canada. andre.roy.chum@ssss.gouv.qc.ca
Telephone: + 1-514-8908000-36146
Fax: +1-514-4127380
Received: April 10, 2007
Revised: December 12, 2007
Published online: February 28, 2008
Abstract

Von Hippel-Lindau syndrome (VHL) is a rare autosomal-dominant, inherited familial cancer syndrome. Hemangioblastomas, pheochromocytomas and renal carcinoma are the frequent reported VHL tumors. Neuroendocrine tumors have also been described, mostly in the pancreas and rarely in the biliary trees. We report the second case of bile duct carcinoid in a 31-year-old VHL woman. She underwent right adrenalectomy for a pheochromocytoma in the past. She also had a positive family history of phenotypic expression of VHL syndrome. The patient presented with biliary colic. Endoscopic retrograde cholangio-pancreatography showed intra-luminal bile duct mass. Surgical exploration identified a beige nodular lesion that was a carcinoid tumor on histology. This new association should be clarified by further genetic investigations.

Keywords: Carcinoid tumor, Bile duct tumor, Von Hippel-Lindau syndrome