Cholangiocarcinoma 2020: the next horizon in mechanisms and management

作者：Jesús M. Bañales, José J.G. Marı́n, Ángela Lamarca, Pedro M. Rodrigues, Shahid A. Khan, Lewis R. Roberts, Vincenzo Cardinale, Guido Carpino, Jesper B. Andersen, Chiara Braconi, Diego F. Calvisi, María J. Perugorria, Luca Fabris, Luke Boulter, Rocı́o I.R. Macı́as, Eugenio Gaudio, Domenico Alvaro, Sergio A. Gradilone, Mario Strazzabosco, Marco Marzioni, Cédric Coulouarn, Laura Fouassier, Chiara Raggi, Pietro Invernizzi, Joachim C. Mertens, Anja Moncsek, Sumera I. Ilyas, Julie K. Heimbach, Bas Groot Koerkamp, Jordi Bruix, Alejandro Forner, John Bridgewater, Juan W. Valle, Gregory J. Gores · 发表于：Nature Reviews Gastroenterology & Hepatology · 年份：2020 · DOI：10.1038/s41575-020-0310-z · 被引用次数：2543 · 研究领域：Cholangiocarcinoma and Gallbladder Cancer Studies、Pancreatic and Hepatic Oncology Research、Cancer Mechanisms and Therapy

Cholangiocarcinoma (CCA) includes a cluster of highly heterogeneous biliary malignant tumours that can arise at any point of the biliary tree. Their incidence is increasing globally, currently accounting for ~15% of all primary liver cancers and ~3% of gastrointestinal malignancies. The silent presentation of these tumours combined with their highly aggressive nature and refractoriness to chemotherapy contribute to their alarming mortality, representing ~2% of all cancer-related deaths worldwide yearly. The current diagnosis of CCA by non-invasive approaches is not accurate enough, and histological confirmation is necessary. Furthermore, the high heterogeneity of CCAs at the genomic, epigenetic and molecular levels severely compromises the efficacy of the available therapies. In the past decade, increasing efforts have been made to understand the complexity of these tumours and to develop new diagnostic tools and therapies that might help to improve patient outcomes. In this expert Consensus Statement, which is endorsed by the European Network for the Study of Cholangiocarcinoma, we aim to summarize and critically discuss the latest advances in CCA, mostly focusing on classification, cells of origin, genetic and epigenetic abnormalities, molecular alterations, biomarker discovery and treatments. Furthermore, the horizon of CCA for the next decade from 2020 onwards is highlighted.