Published online Dec 15, 2021. doi: 10.4251/wjgo.v13.i12.1939
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.
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.