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Canadian Journal of Gastroenterology
Volume 19, Issue 12, Pages 731-733
Brief Communication

Well Differentiated Intrahepatic Cholangiocarcinoma in the Setting of Biliary Papillomatosis: A Case Report and Review of the Literature

Heather Cox,1 Michael Ma,2 Ronald Bridges,2 Estifanos Debru,1 Oliver Bathe,1 Francis Sutherland,1 and Elijah Dixon1

1Department of Surgery, University of Calgary, Calgary, Alberta, Canada
2Division of Gastroenterology, Department of Medicine, University of Calgary, Calgary, Alberta, Canada

Received 19 June 2005; Accepted 29 June 2005

Copyright © 2005 Hindawi Publishing Corporation. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


A 64-year-old man presented with long-standing, vague, epigastric abdominal pain. History, physical examination and laboratory studies were noncontributory. However, serial computed tomography scans revealed a rapidly progressive mass in segment 2 of the liver. Surprisingly, surgical pathology revealed a well-differentiated intrahepatic cholangiocarcinoma associated with biliary papillomatosis (BP). BP is a rare, benign and potentially fatal disease of the intra- and extrahepatic bile ducts. It is typified by numerous multicentric papillary fronds arising from biliary columnar epithelium. Most patients present with symptoms of jaundice and cholangitis. Although a benign disease, a review of the literature demonstrated that BP often recurs after surgical resection, carries a poor prognosis and has a moderately high malignant transformation rate. Treatment options for BP include surgical resection, transplant, ablation, stenting and/or bypass.