Korean J Hepatobiliary Pancreat Surg.
2010 Mar;14(1):57-62.
Double Intrahepatic and Extrahepatic Cholangiocarcinomas Arising from Biliary Papillomatosis: A Case Report
- Affiliations
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- 1Department of Surgery, Deajeon St. Mary's Hospital, The Catholic University of Korea College of Medicine, Korea. sejoonkim@hanmail.net
- 2Department of Pathology, Deajeon St. Mary's Hospital, The Catholic University of Korea College of Medicine, Korea.
Abstract
- Biliary papillomatosis is a rare disease entity characterized by multiple papillary adenomas along the bile duct mucosa. It is widely accepted that the adenoma-carcinoma sequence represents the process by which most, if not all, cholangiocarcinomas of an intraductal-growing type arise. Interestingly, friable papillary projections easily detached from the primary site can be implanted into the other sites in the bile duct in suitable animal models, resulting in multiple tumors. A 76-year-old male was referred to our hospital due to intermittent abdominal discomfort. Imaging workups revealed two lesions: wall thickening in the proximal portion of the left interhepatic duct and abrupt narrowing of the distal common bile duct. A hepatopancreaticoduodenectomy was carried out and pathological analysis demonstrated a well-differentiated adenocarcinoma of the left hepatic duct and carcinoma in situ of the distal common bile duct on a background of biliary papillomatosis. Six days after the operation, the patient received a re-exploration due to ruptured mesenteric vessels. Unfortunately, 3 months after the initial operation, the patient died of aspiration pneumonia. Biliary papillomatosis can present with a broad spectrum of disease characteristics ranging from adenoma to, usually, low-grade adenocarcinoma. If there is a complete excision, and a favorable postoperative course is secured, excellent survival can be expected after surgery.
