Copyright ©The Author(s) 2021. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastrointest Oncol. Dec 15, 2021; 13(12): 1939-1955
Published online Dec 15, 2021. doi: 10.4251/wjgo.v13.i12.1939
Moving forward in the treatment of cholangiocarcinoma
Tommaso M Manzia, Alessandro Parente, Ilaria Lenci, Bruno Sensi, Martina Milana, Carlo Gazia, Alessandro Signorello, Roberta Angelico, Giuseppe Grassi, Giuseppe Tisone, Leonardo Baiocchi
Tommaso M Manzia, Bruno Sensi, Carlo Gazia, Roberta Angelico, Giuseppe Tisone, Hepato-Pancreato-Biliary and Transplant, Department of Surgery, University of Rome Tor Vergata, Rome 00133, Italy
Alessandro Parente, The Liver Unit, Queen Elizabeth Hospital Birmingham, Birmingham B15 2TH, United Kingdom
Ilaria Lenci, Martina Milana, Alessandro Signorello, Giuseppe Grassi, Leonardo Baiocchi, Hepatology Unit, University of Tor Vergata, Rome 00133, Italy
Author contributions: Manzia TM contributed to acquisition of data, analysis and interpretation, drafting of manuscript and critical revision; Parente A, Lenci I, Sensi B, Milana M, Gazia C, Signorello A, Angelico R, Grassi G and Tisone G contributed to acquisition of data and critical revision; Baiocchi L contribute to proposal of study, study conception, correction of manuscript and critical revision.
Conflict-of-interest statement: The authors declare no conflict of interest.
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: Leonardo Baiocchi, MD, PhD, Associate Professor, Hepatology Unit, University of Tor Vergata, Viale Oxford 31, Rome 00133, Italy.
Received: March 16, 2021
Peer-review started: March 16, 2021
First decision: May 3, 2021
Revised: May 14, 2021
Accepted: October 14, 2021
Article in press: October 14, 2021
Published online: December 15, 2021

Despite being the second most frequent primary liver tumor in humans, early diagnosis and treatment of cholangiocarcinoma (CCA) are still unsatisfactory. In fact, survival after 5 years is expected in less than one fourth of patients diagnosed with this disease. Rare incidence, late appearance of symptoms and heterogeneous biology are all factors contributing to our limited knowledge of this cancer and determining its poor prognosis in the clinical setting. Several efforts have been made in the last decades in order to achieve an improved classification/understanding with regard to the diverse CCA forms. Location within the biliary tree has helped to distinguish between intrahepatic, perihilar and distal CCA types. Sequence analysis contributed to identifying several characteristic genetic aberrations in CCA that may also serve as possible targets for therapy. Novel findings are expected to significantly improve the management of this malignancy in the near future. In this changing scenario our review focuses on the current and future strategies for CCA treatment. Both systemic and surgical treatments are discussed in detail. The results of the main studies in this field are reported, together with the ongoing trials. The current findings suggest that an integrated multidisciplinary approach to this malignancy would be helpful to improve its outcome.

Keywords: Cholangiocarcinoma, Treatment, Genetic aberration, Immunotherapy, Liver resection, Liver transplantation

Core Tip: Cholangiocarcinoma is a lethal malignancy characterized by a poor survival. In this review we discuss in detail the actual treatment and the future therapeutic perspectives for this cancer. Systemic and surgical strategies are reported with the corresponding results. Improved knowledge of this malignancy and a multidisciplinary therapeutic approach are likely to improve the cholangiocarcinoma outcome in the future.