Background/purpose: Cholangiocarcinoma (CCC) is a rare malignant tumor of the biliary system with a poor prognosis that frequently presents in advanced unresectable stages. A discussion of the role of liver transplantation (LTx) for intrahepatic (IH)-CCC will be presented, based on a review of the international literature.
Methods: A search of the international literature related to the topic was performed in the established online databases and databanks. Only those reports with patient series larger than ten were included.
Results: The largest series of patients with IH-CCC ( n = 186) was reported in the Data Analysis Booklet of the European Liver Transplant Registry (ELTR), revealing 1-, 3-, 5-, 8-, and 10-year survival rates of 58%, 38%, 29%, 23%, and 21%. Other single-center series had comparable or even worse outcomes. More recent experience identified a certain highly selected group of patients who could benefit from LTx in an otherwise unresectable state of disease. Several single-center studies found tumor stage or size, lymph node involvement, and positive surgical margins/contiguous organ infiltration to be predictors of outcome. The recently published Mayo Clinic protocol uses preoperative chemoirradiation and staging at laparotomy before proceeding to liver transplantation, resulting in an early experience with 100% 1-year survival.
Conclusions: Generally speaking, IH-CCC is not a favorable indication for liver transplantation. However, there is a highly selected group of patients with early tumor stages potentially benefitting from LTx. Combination with neoadjuvant chemoirradiation may further improve results after LTx. Living-donation LTx may open new therapeutic possibilities to treat patients at an early stage of disease.