Key Points

1. Resection rates for cholangiocarcinoma (unrelated to primary sclerosing cholangitis) have increased to 54% to 79%, and the subsequent 5-year survival rates are 24% to 31%.

2. Multimodality approaches involving various combinations of chemotherapy, irradiation, and surgery increasingly are being used to treat cholangiocarcinoma.

3. The role of liver transplantation in the management of cholangiocarcinoma is limited by the perception that it is inappropriate to use scarce organs when 5-year survival rates are 25%.

4. Liver transplantation is an important intervention in patients with tumors that remain unresectable after chemotherapy. The role of liver transplantation in patients with extrahepatic disease that responds to chemotherapy is controversial. Careful timing of surgery is required to avoid secondary drug resistance.

5. Liver transplantation has been successfully applied to a range of rare hepatic malignancies, but small numbers preclude strong recommendations on the appropriateness of this practice.