Cholangiocarcinomas (CCAs) are malignant tumors that can develop anywhere along the biliary tree. Almost 10% of cholangiocarcinomas arise from the intra-hepatic bile ducts (iCCA); 50–60% from the bifurcation of the hepatic duct (perhilar cholangiocarcinoma, pCCA); and 20–30% from the distal bile duct (dCCA). The 7th edition of the AJCC staging system, released in 2010, divides the tumors into two major categories: perihilar (pCCA) and distal (dCCA) cholangiocarcinoma, given the differences in anatomy of the bile duct and consideration of local factors related to resectability. There are separate histological classifications for intrahepatic and extrahepatic cholangiocarcinoma. The majority of CCAs (90%) are well or moderately differentiated adenocarcinomas. Other features include invasiveness with early neural, perineural, periductal and lymphatic infiltration (more than 50% of cases at diagnosis) and longitudinal subepithelial infiltration along the wall of the bile duct up to 2 cm proximally and 1 cm distally. In this chapter the extrhepatic bile duct cancers are analyzed.
Part of the book: Bile Duct Cancer