Hilar cholangiocarcinoma.

Optimal treatment of hilar cholangiocarcinoma depends on location of the cancer and extent of biliary and vascular involvement. Candidates for resection or transplantation must be evaluated and managed by a multidisciplinary team at a high-volume hepatobiliary center. Success requires absence of distant nodal or extrahepatic metastases and an adequate functional liver remnant with a negative ductal margin. Ipsilateral portal vein resection and reconstruction should be performed in patients with venous involvement. Neoadjuvant chemoradiation and liver transplantation is the best treatment option for patients with unresectable hilar cholangiocarcinoma without nodal or distant metastases and for patients with underlying chronic liver disease.