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Cholangiocarcinoma 2020: the next horizon in mechanisms and management.


ABSTRACT: 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.

SUBMITTER: Banales JM 

PROVIDER: S-EPMC7447603 | biostudies-literature | 2020 Sep

REPOSITORIES: biostudies-literature

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Cholangiocarcinoma 2020: the next horizon in mechanisms and management.

Banales Jesus M JM   Marin Jose J G JJG   Lamarca Angela A   Rodrigues Pedro M PM   Khan Shahid A SA   Roberts Lewis R LR   Cardinale Vincenzo V   Carpino Guido G   Andersen Jesper B JB   Braconi Chiara C   Calvisi Diego F DF   Perugorria Maria J MJ   Fabris Luca L   Boulter Luke L   Macias Rocio I R RIR   Gaudio Eugenio E   Alvaro Domenico D   Gradilone Sergio A SA   Strazzabosco Mario M   Marzioni Marco M   Coulouarn Cédric C   Fouassier Laura L   Raggi Chiara C   Invernizzi Pietro P   Mertens Joachim C JC   Moncsek Anja A   Rizvi Sumera S   Heimbach Julie J   Koerkamp Bas Groot BG   Bruix Jordi J   Forner Alejandro A   Bridgewater John J   Valle Juan W JW   Gores Gregory J GJ  

Nature reviews. Gastroenterology & hepatology 20200630 9


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  ...[more]

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