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Langenbeck's Archives of Surgery
Published in: Langenbeck's Archives of Surgery 1/2009

01-01-2009 | New Surgical Horizons

Pancreaticobiliary maljunction and carcinogenesis to biliary and pancreatic malignancy

Authors: Takahiko Funabiki, Toshiki Matsubara, Shuichi Miyakawa, Shin Ishihara

Published in: Langenbeck's Archives of Surgery | Issue 1/2009

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Abstract

Background

It is widely accepted that congenital choledochal cyst is associated with pancreaticobiliary maljunction (PBM). But, PBM is an independent disease entity from choledochal cyst. PBM is synonymous with “abnormal junction of the pancreaticobiliary ductal system”, “anomalous arrangement of pancreaticobiliary ducts”, “anomalous union of bilio-pancreatic ducts”, etc. Cases with PBM not associated with biliary duct dilatation are often found, and these cases are frequently complicated gallbladder cancer. The Japanese Study Group of Pancreaticobiliary Maljunction was started in 1983, and defined diagnostic criteria and nationwide registration system of PBM cases was started. PBM is defined as a union of the pancreatic and biliary ducts which is located outside the duodenal wall. Bile and pancreatic juice reflux and regurgitate mutually.

Biliary carcinogenesis

The most bothersome problem is biliary carcinogenesis. Gallbladder cancers arise in 14.8% and bile duct cancers arise in 4.9%. The incidence of the gallbladder carcinoma of PBM without bile duct dilatation is 36.1%. Many investigators have tried to clarify the carcinogenic process, from various aspects. The biliary epithelia are injured by harmful substances, and in the course of repair, multiple alterations of oncogenes and tumor suppressor genes are followed, and they lead to carcinoma through multistage interaction. In the biliary epithelia of PBM, incidence and degree of hyperplasia are characteristic. K-ras gene mutations are observed in the cancerous as well as noncancerous lesions of biliary tract of PBM patients. Mutations of p53 gene and overexpression of p53 protein are also found in the cancerous and noncancerous lesions. These changes are called “hyperplasia–carcinoma sequence”.

Treatment

Total excision of the extrahepatic bile duct with gallbladder followed by hepaticojejunostomy, Roux-en-Y, or end-to-side hepaticoduodenostomy are treatment of choice, even for cases with not dilated bile duct, because the incidence of cancer in the nondilated bile duct is not negligible, and genetic changes are seen in a nondilated bile duct.
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Metadata
Title
Pancreaticobiliary maljunction and carcinogenesis to biliary and pancreatic malignancy
Authors
Takahiko Funabiki
Toshiki Matsubara
Shuichi Miyakawa
Shin Ishihara
Publication date
01-01-2009
Publisher
Springer-Verlag
Published in
Langenbeck's Archives of Surgery / Issue 1/2009
Print ISSN: 1435-2443
Electronic ISSN: 1435-2451
DOI
https://doi.org/10.1007/s00423-008-0336-0

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