Cholangiocarcinoma

Cholangiocarcinoma is a malignant tumor originating from the epithelial lining of the bile duct. It is classified into three anatomically and clinically distinct subtypes - intrahepatic, perihilar, and distal - based on its location within the biliary tree.

It may be asymptomatic in the early stages, with classic symptoms of painless jaundice, weight loss, and abdominal pain usually appearing in advanced disease.

Imaging alone is not sufficient to make a diagnosis. Pathologic diagnosis of operative specimens is required for a definitive diagnosis.

Management is guided by tumor location and stage at presentation. Surgical resection remains the only potentially curative treatment for localized disease, while unresectable or metastatic disease is typically treated with chemotherapy plus immunotherapy, with locoregional, transplant, and supportive therapies considered for selected patients. Participation in clinical trials should be considered.

Cholangiocarcinomas are cancers arising from the bile duct epithelium. More than 95% are adenocarcinomas. These can be divided depending on their location in the biliary tree: intrahepatic or extrahepatic. Intrahepatic cholangiocarcinomas arise within the liver parenchyma. Extrahepatic cholangiocarcinomas occur outside the liver, within the bile ducts, and are further categorized into perihilar and distal. Perihilar cholangiocarcinomas, also known as Klatskin tumors, are the most common type of extrahepatic cholangiocarcinomas that are located at the junction of the right and left hepatic ducts. Distal cholangiocarcinomas arise in the extrahepatic bile duct above the ampulla of Vater.[1]National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: biliary tract cancers [internet publication]. https://www.nccn.org/guidelines/category_1 Extrahepatic cholangiocarcinomas are more common than intrahepatic cholangiocarcinomas.[1]National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: biliary tract cancers [internet publication]. https://www.nccn.org/guidelines/category_1 Mixed hepatocellular carcinoma-cholangiocarcinoma is a rare variant that occurs in an estimated 1% to 10% of primary liver cancers and is not covered in this topic.[1]National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: biliary tract cancers [internet publication]. https://www.nccn.org/guidelines/category_1