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  • Cholangiocarcinoma refers to malignant tumors of the biliary tree which can occur within or outside of the liver although only intrahepatic tumors are discussed here. Although technically considered cholangiocarcinomas, extrahepatic neoplasms of the bile ducts are covered in extrahepatic biliary cancers.
  • The cell of origin of cholangiocarcinoma is the biliary epithelial cell. Although the pathogenesis of this disease is not well-understood several risk factors have been identified. These include a history of primary sclerosing cholangitis or chronic infection of the biliary tree with certain liver flukes. Additionally, previous usage of "Thorotrast", a biliary contrast reagent for radiographic procedures, is an important risk factor. Note that there is not a strong correlation with risk factors of Hepatocellular Carcinoma such as HBV infection or history of cirrhosis.
Morphology and Clinical Consequences
  • Like all tumors of the biliary tree, these neoplasms are adenocarcinomas. Sadly, cholangiocarcinomas possess a dismal prognosis with survival rates of 1 to 2 years.