title: Carcinoid tumor of the common bile duct: a rare complication of von Hippel-Lindau syndrome.
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.
authors.affiliation: Department of Surgery, Saint-Luc Hospital, CHUM, 1058 Saint-Denis Street, Montreal, Quebec H2X 3J4, Canada. andre.roy.chum@ssss.gouv.qc.ca.
authors.forename: Otmane
authors.initials: O
authors.lastname: Nafidi
authors.forename: Bich-N
authors.initials: BN
authors.lastname: Nguyen
authors.forename: André
authors.initials: A
authors.lastname: Roy
mesh_terms: D000328:Adult; D001650:Bile Duct Neoplasms / Q000175:diagnosis*; D001652:Bile Ducts / Q000473:pathology / Q000601:surgery; D002276:Carcinoid Tumor / Q000175:diagnosis*; D049109:Cell Proliferation; D004724:Endoscopy; D005260:Female; D006801:Humans; D007249:Inflammation; D010641:Phenotype; D010751:Phosphopyruvate Hydratase / Q000378:metabolism; D013577:Syndrome; D016896:Treatment Outcome; D006623:von Hippel-Lindau Disease / Q000378:metabolism*
chemical_list: D010751:Phosphopyruvate Hydratase