Published online Jan 27, 2022. doi: 10.4240/wjgs.v14.i1.1
Peer-review started: April 7, 2021
First decision: June 23, 2021
Revised: July 12, 2022
Accepted: December 28, 2021
Article in press: December 28, 2021
Published online: January 27, 2022
Cholangiocarcinoma (CCA) is the second most common liver cancer with a median survival of 12-24 mo without treatment. It is further classified based on its location into intrahepatic CCA (iCCA), perihilar CCA (pCCA), and distal CCA. Surgical resection is the mainstay of treatment, but up to 70% of these tumors are inoperable at the time of diagnosis. CCA was previously an absolute contraindication for liver transplantation (LT) due to poor outcomes primary due to early recurrent disease. However, improvement in patient selection criteria and neoadjuvant treatment protocols have improved outcomes for inoperable pCCA patients with recent studies reporting LT may improve survival in iCCA. Future advances in the treatment of CCA should include refining patient selection criteria and organ allocation for all subtypes of CCA, determining effective immunotherapies and the evolving role of personalized medicine in patients ineligible for surgical resection or LT. Our article reviews the current status of LT in CCA, along with future directions in managing patients with CCA.
Core Tip: Perihilar cholangiocarcinoma (pCCA) is an accepted indication for liver transplantation (LT) using a strict selection process and standardized neoadjuvant treatment protocol with pre-operative disease staging. Intrahepatic cholangiocarcinoma (iCCA) has historically been a contraindication for LT due to poor reported outcomes. With improved tumor detection, patient selection, and neoadjuvant treatment, recent studies have reported improved survival in iCCA patients with LT. No standardized protocol exists for the treatment of iCCA using LT. Our review analyzes the history and current literature on the treatment of pCCA and iCCA, along with gaps in knowledge and future perspectives.