Cholangiocarcinoma

Classic symptoms of cholangiocarcinoma - including painless jaundice, weight loss, and abdominal pain - usually appear in advanced disease.

Liver enzymes, blood levels of carbohydrate antigen (CA) 19-9, carcinoembryonic antigen, CA-125; abdominal ultrasound, abdominal CT/MRI, MR angiography, and cholangiography are used for evaluation.

Surgical resection offers the only potential cure for early-stage disease.

Chemotherapy may have a positive effect on overall survival of patients following resection of intrahepatic cholangiocarcinoma.

Liver transplant is indicated in a small subset of patients.

Cholangiocarcinomas are cancers arising from the bile duct epithelium. These can be divided depending on their location in the biliary tree: intrahepatic or extrahepatic (perihilar and distal). Perihilar tumors involving the bifurcation of the ducts are also known as Klatskin tumors. More than 95% are adenocarcinomas. Most are of the infiltrating nodular or diffusely infiltrating type. Purely nodular or papillary are less frequent subtypes.