Cholangiocarcinoma 2020 the next horizon in mechanisms and management


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 classifi

Conflicts of Interest

Lorenza Rimassa received consulting fees from AbbVie, Astra- Zeneca, Basilea, Bayer, BMS, Elevar Therapeutics, Exelixis, Genenta, Hengrui, Incyte, Ipsen, IQVIA, Jazz Pharmaceuticals, MSD, Nerviano Medical Sciences, Roche, Servier, Taiho Oncology, Zymeworks. Lecture fees from AstraZeneca, Bayer, BMS, Guerbet, Incyte, Ipsen, Roche, Servier. Travel expenses from AstraZeneca. Research grants (to Institution) from Agios, AstraZeneca, BeiGene, Eisai, Exelixis, Fibrogen, Incyte, Ipsen, Lilly, MSD, Nerviano Medical Sciences, Roche, Servier, Taiho Oncology, TransThera Sciences, Zymeworks. All other authors declare no conflict of interest.

Author Contributions

Conceptualization: VZ, GT, EV, LR

Methodology: VZ, GT, EV, LR

Supervision: LR

Visualization: VZ, GT

Writing - original draft: VZ, GT, EV, LR

Writing - review & editing: VZ, GT, EV, LR

Approval of final manuscript: all authors


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Banales, JM., Marin, JJG., LaMarca, A., Rodrigues, PM., Khan, SA., Roberts, LR., Cardinale, V., Carpino, G., Andersen, JB., Braconi, C., Calvisi, DF., Perugorria, MJ., Fabris, L., Boulter, L., Macias, RIR., Gaudio, E., Alvaro, D., Gradilone, SA., Strazzabosco, M., ... Gores, GJ. (2020). Cholangiocarcinoma 2020: the next horizon in mechanisms and management. Nature Reviews Gastroenterology and Hepatology, 17(9), 557-588. https://doi.org/10.1038/s41575-020-0310-z

@article{efa67240dc6c4135bf82fc55926f9cdc,

title = "Cholangiocarcinoma 2020: the next horizon in mechanisms and management",

author = "JM Banales and JJG Marin and A LaMarca and PM Rodrigues and SA Khan and LR Roberts and V Cardinale and G Carpino and JB Andersen and C Braconi and DF Calvisi and MJ Perugorria and L Fabris and L Boulter and RIR Macias and E Gaudio and D Alvaro and SA Gradilone and M Strazzabosco and M Marzioni and C Coulouarn and L Fouassier and C Raggi and P Invernizzi and JC Mertens and A Moncsek and S Rizvi and J Heimbach and \{Groot Koerkamp\}, B and J Bruix and A Forner and J Bridgewater and JW Valle and GJ Gores",

Cholangiocarcinoma 2020: the next horizon in mechanisms and management

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 first liver cancers and 3% of gastrointestinal malignancies.

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The silent presentation of these tumours united with their highly aggressive innateness and refractoriness to chemotherapy contribute to their alarming mortality, showing 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 free therapies. In the past decade, increasing efforts have been made to understand the complexity of these tumours and to progress new diagnostic tools and therapies that might help to upgrade patient outcomes. In this specialist Consensus Statement, which is endorsed by the European Network for the Study of Cholangiocarcinoma, we aim cholangiocarcinoma 2020 the next horizon in mechanisms and management

Cholangiocarcinoma 2020: the next horizon in mechanisms and management.

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Journal article
  • Banales JMDepartment of Liver and Gastrointestinal Diseases, Biodonostia Health Research Institute - Donostia University Hospital, University of the Basque Country (UPV/EHU), San Sebastian, Spain. jesus.banales@biodonostia.org.
  • Marin JJGNational Institute for the Study of Liver and Gastrointestinal Diseases (CIBERehd, "Instituto de Salud Carlos III"), San Sebastian, Spain.
  • Lamarca ADepartment of Medical Oncology, The Christie NHS Foundation Trust, Manchester, UK.
  • Rodrigues PMDepartment of Liver and Gastrointestinal Diseases, Biodonostia Health Research Institute - Donostia University Hospital, University of the Basque Country (UPV/EHU), San Sebastian, Spain.
  • Khan SADepartment of Surgery and Cancer, Imperial College London, Hammersmith Hospital, London, UK.
  • Roberts LRDivision of Gastroenterology and Hepatology, Mayo Clinic College of Medicine and Science, Rochester, MN, USA.
  • Cardinale VDepartment of Medico-Surgical Sciences and Biotechnologies, Sapienza University of Rome, Rome, Italy.
  • Carpino GDepartment of Movement, Human and Hea