Carcinomas of the Gallbladder and the Extrahepatic Bile Ducts - Biology and Pathology Cancer of the biliary tract, including gallbladder and bile duct carcinoma, accounts for approximately 2% of all malignancies, with increasing incidence. Biliary tract cancers are a consequence of a stepwise malignant transformation of the biliary epithelium. Cholangiocarcinoma located at the right and left hepatic ducts or near their junction is called hilar cholangiocarcinoma or Klatskin tumor and is considered as an extrahepatic lesion(carcinoma of the extrahepatic bile ducts). Cancer of extrahepatic bile ducts may also arise in the cystic duct or choledochal duct as well as in the gallbladder. The UICC TNM staging systems of carcinomas of the gallbladder and extrahepatic bile ducts differs from those of primary liver tumors. Prognosis of patients is poor. Although many etiological factors have been characterized,the cause of carcinomas of the gallbladder and extrahepatic bile ducts remains speculative in most cases. Chronic inflammation due to sclerosing cholangitis or due to biliary cysts as a part of ductal plate malformation is associated with carcinogenesis. Histopathologically,the vast majority of biliary cancers are adenocarcinomas. Other types of carcinomas including neuroendocrine tumors are rare. The natural history of the carcinogenic process of the biliary tract is a multistep process and is described as intraepithelial neoplasiacarcinoma sequence with underlying accumulation of genetic alterations.

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2004
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