Cholangiocarcinoma is a rare cancer found in the tissue of the bile ducts which drain bile from the liver into the small intestine. Tumors produce symptoms by blocking the bile ducts. Cholangiocarcinoma has an annual incidence of 1–2 cases per 100,000 in the Western world, but rates of cholangiocarcinoma have been rising worldwide over the past several decades.
Common symptoms may include:
Cholangiocarcinoma can affect any area of the bile ducts, either within or outside the liver. Intrahepatic tumors occur in the bile ducts within the liver. Those occurring in the ducts outside the liver are extrahepatic. Tumors occurring at the site where the bile ducts exit the liver are referred to as perihilar. A cholangiocarcinoma occurring at the junction where the left and right hepatic ducts meet to form the common bile duct are referred to as a Klatskin tumor.
Cholangiocarcinoma is often in an advanced stage by the time symptoms develop, which may limit treatment options.
There is no potentially curative treatment except surgery where all tumors have to be cut out surgically (resection). Patients with cholangiocarcinoma are generally managed, though never cured, with chemotherapy or radiation therapy as well as palliative care measures, and these are also used as adjuvant therapies post-surgically in cases where resection has been successful.
Surgical resection offers the only potential chance of cure in cholangiocarcinoma. The median duration of survival is less than 6 months in inoperable, untreated, otherwise healthy patients with tumors involving the liver by way of the intrahepatic bile ducts and hepatic portal vein.