Abstract

Biliary tract cancers arise in the biliary tract epithelium and include intrahepatic cholangiocarcinoma, perihilar cholangiocarcinoma, distal extrahepatic cholangiocarcinoma, and gallbladder cancer. These sites are anatomically contiguous and may appear related, but they are heterogeneous with distinct genetic and molecular signatures. Cholangiocarcinoma is the malignant tumor arising from the epithelium of the biliary tract, first histologically described by Durand-Fardel in 1840. Classification as originating from the intra- and extrahepatic biliary epithelium1 is a common way of classifying these tumors as prognosis and behavior are different in both types. Extrahepatic cholangiocarcinomas are further separated like the Bismuth classification; into upper-third or perihilar (including the confluence of the right and left biliary ducts) tumors; middle-third tumors; and distal bile duct tumors. Performance of a marginnegative resection with regional lymphadenectomy remains the cornerstone of successful treatment of cholangipcarcinoma.

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