Bile duct cancers are almost always histologically adenocarcinoma, known as cholangiocarcinoma. Risk factors associated with the disease are: the presence of choledochal cysts and Caroli's disease, infestation with Clonorchis sinensis, hepatolithiasis, sclerosing cholangitis, ulcerative colitis, and exposure to Thorotrast.

The primary tumor may occur in three locations; one-third in the distal common bile duct, one-third in the common hepatic duct, and one-third in the right or left hepatic duct. When the tumor is located at the hepatic artery bifurcation, it is referred to as a Klatskin tumor.

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