Cholangiocarcinoma

Cholangiocarcinoma may be asymptomatic in the early stages. Classic symptoms of 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, and endoscopic ultrasound are used for evaluation.

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

Chemotherapy with or without immunotherapy may have a positive effect on overall survival of patients following resection of 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.