Cholangiocarcinomas are epithelial neoplasms that originate from cholangiocytes and can occur at any level of the biliary tree. They are broadly classified into intrahepatic tumours, (extrahepatic) hilar tumours and (extrahepatic) distal bile duct tumours. In spite of well-understood predispositions, most cholangiocarcinomas arise in the absence of risk factors. In suspected cases, the diagnosis can be established with non-invasive imaging studies.
Biliary invasion should be reserved for patients with obstruction. In high-risk patients, advanced cytological tests of aneuploidy (digital image analysis and fluorescent in situ hybridization) aid early diagnosis. In the absence of primary sclerosing cholangitis, curative surgical resection has 5-year survival rates of 2–43%, higher survival observed in patients with clear surgical margins and concomitant hepatic resection for hilar tumours.
Patients with unresectable cholangiocarcinoma or pre-existing primary sclerosing cholangitis should be considered for liver transplantation with neoadjuvant chemoirradiation, in specialized centres.