Copyright ©The Author(s) 2021. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Hepatol. Sep 27, 2021; 13(9): 1003-1018
Published online Sep 27, 2021. doi: 10.4254/wjh.v13.i9.1003
Advances in the management of cholangiocarcinoma
Andreas G Zori, Dennis Yang, Peter V Draganov, Roniel Cabrera
Andreas G Zori, Dennis Yang, Peter V Draganov, Roniel Cabrera, Division of Gastroenterology, Hepatology, and Nutrition, University of Florida, Gainesville, FL 32608, United States
Author contributions: Zori AG, Yang D, Draganov PV, and Cabrera R contributed to conception and design of the paper; Draganov PV and Cabrera R revised paper critically for important intellectual content; Zori AG, Yang D wrote majority of the draft;
Conflict-of-interest statement: Zori AG, Draganov PV, and Cabrera R have no conflicts of interest; Yang D is a consultant for Lumendi and Boston Scientific.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:
Corresponding author: Andreas G Zori, MD, Assistant Professor, Division of Gastroenterology, Hepatology, and Nutrition, University of Florida, 1329 SW 16th St Suite 5251, Gainesville, FL 32608, United States.
Received: February 26, 2021
Peer-review started: February 26, 2021
First decision: May 3, 2021
Revised: June 9, 2021
Accepted: August 6, 2021
Article in press: August 6, 2021
Published online: September 27, 2021

Cholangiocarcinoma (CCA) is a primary malignancy of the bile ducts with three anatomically and molecularly distinct entities: Intrahepatic CCA (iCCA), perihilar CCA (pCCA), and distal CCA. As a result of phenotypic and anatomic differences they differ significantly with respect to management. For each type of CCA there have been significant changes in management over the last several years which will be discussed in this review. Although resection remains the standard of care for all types of CCA, liver transplantation has been established as curative treatment for selected patients with pCCA and is being evaluated for iCCA with early success. With respect to systemic therapy capecitabine is now first line adjuvant therapy for all biliary tract malignancies after curative intent resection. Progress in exploiting the pathologic mutations and molecular abnormalities has also yielded regulatory approval of targeted therapy for CCA in patients with acquired alterations in the fibroblast growth factor receptor. There is also increased consensus in managing malignant biliary obstruction associated with CCA where pre-operative biliary stenting is not beneficial while self-expanding metal stents have been shown to be superior to plastic stents in patients who are not surgical candidates.

Keywords: Cholangiocarcinoma, Intrahepatic cholangiocarcinoma, Perihilar cholangiocarcinoma, Liver transplantation, Chemotherapy

Core Tip: This review presents recent advances in the management of cholangiocarcinoma with particular focus on the expanding role for liver transplantation, updated guidelines in the use of chemotherapy, novel applications of individualized therapy targeting the specific mutation profile of tumors, and management of malignant biliary obstruction.