Journal
NATURE REVIEWS GASTROENTEROLOGY & HEPATOLOGY
Volume 17, Issue 9, Pages 557-588Publisher
NATURE PORTFOLIO
DOI: 10.1038/s41575-020-0310-z
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Categories
Funding
- Spanish Ministry of Economy and Competitiveness [FIS PI12/00380, FIS PI15/01132, FIS PI18/01075, CON14/00129, FIS PI14/00399, FIS PI17/00022, RYC-2015-17755]
- Fondo Europeo de Desarrollo Regional (FEDER)
- ISCIII CIBERehd
- Diputacion Foral de Gipuzkoa [DFG15/010, DFG16/004]
- BIOEF (Basque Foundation for Innovation and Health Research: EiTB Maratoia) [BIO15/CA/016/BD]
- Department of Health of the Basque Country [2015111100, 2017111010]
- Fundacion Cientifica de la Asociacion Espanola Contra el Cancer (AECC Scientific Foundation)
- European Commission Horizon 2020 programme (ESCALON project) [825510]
- Danish Medical Research Council
- Danish Cancer Society
- Novo Nordisk Foundation
- A.P. MOller Foundation
- Carlos III Institute of Health, Spain [PI16/00598, PI18/00428]
- European Regional Development Fund
- Ministry of Science and Innovation, Spain [SAF2016-75197-R]
- Asociacion Espanola Contra el Cancer, Spain (AECC-2017)
- Centro Internacional sobre el Envejecimiento, Spain (OLD-HEPAMARKER) [0348-CIE-6-E]
- Christie Charity
- Universita Politecnica delle Marche, Ancona, Italy [040020_R.SCIENT.A_2018_MARZIONI_M_STRATEGICO_2017]
- Yale Liver Center Clinical and Translational Core and the Cellular and Molecular Core (Silvio O. Conte Digestive Diseases Research Center) [DK034989]
- INSERM
- Universite de Rennes
- INCa
- ITMO Cancer AVIESAN dans le cadre du Plan Cancer (Non-coding RNA in Cancerology: Fundamental to Translational)
- Ligue Contre le Cancer
- Region Bretagne
- Instituto de Salud Carlos III [PI18/00763]
- AECC [PI044031]
- WCR (AICR) [16-0026]
- ISCIII [PI13/01229, PI18/00542]
- Instituto de Salud Carlos III
- EASL Registry Award 2016 (European CCA Registry, ENS-CCA)
- EASL Registry Award 2019 (European CCA Registry, ENS-CCA)
Ask authors/readers for more resources
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 similar to 15% of all primary liver cancers and similar to 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 similar to 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.
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