Journal
WORLD JOURNAL OF GASTROENTEROLOGY
Volume 14, Issue 8, Pages 1299-1301Publisher
W J G PRESS
DOI: 10.3748/wjg.14.1299
Keywords
carcinoid tumor; bile duct tumor; von Hippel-Lindau syndrome
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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 intraluminal 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. (c) 2008 WJG. All rights reserved.
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