Cholangiocarcinoma

Cholangiocarcinoma (CCA) is a heterogeneous group of cancers arising from the epithelial cells lining the bile ducts. It is classified by anatomic location: intrahepatic (within the liver), perihilar (at the liver hilum, also called Klatskin tumors), and distal (in the common bile duct). Each subtype has distinct biology, treatment approaches, and prognosis. CCA is often diagnosed late because early-stage disease produces few symptoms, and bile duct anatomy makes tumors difficult to detect on routine imaging. By the time jaundice or pain develop, the cancer may be locally advanced or metastatic. Surgical resection offers the best chance for cure, but only about 20-30% of patients are candidates for surgery at diagnosis. For advanced disease, gemcitabine-cisplatin chemotherapy has been the standard first-line treatment, with recent trials showing benefit from adding immunotherapy (durvalumab). Genomic profiling has revealed actionable targets in a significant portion of patients, particularly FGFR2 fusions and IDH1 mutations, which now have FDA-approved targeted therapies.