[en] Cholangiocarcinoma (CCA) is a cancer that originates within the bile ducts. Traditionally considered to be a rare neoplasm, increased awareness of CCA alongside advancements in diagnosis and the rising prevalence of certain risk factors have contributed to a global increase in incidence and mortality. CCAs are highly heterogeneous from the clinical, histomorphological and molecular perspectives but commonly share a poor prognosis. These tumours usually develop and progress silently; by the time they are detected, it is often too late for curative surgical intervention. In such cases, current therapeutic approaches offer modest survival improvements and are generally considered palliative. Although well-known risk factors predispose individuals to developing CCA, the majority of cases are considered sporadic, occurring without any identifiable underlying condition. Over the past decade, substantial collaborative efforts have been made to improve our understanding of the aetiopathogenesis of these tumours, aiming to identify novel biomarkers and therapeutic targets to develop more effective treatments. The ultimate goal is to improve patient outcomes and overall well-being. However, there are significant gaps in our understanding of the molecular mechanisms that drive cholangiocarcinogenesis. In this international Consensus Statement, which is endorsed by the European Network for the Study of Cholangiocarcinoma, we provide a critical overview of the latest advancements in the field of CCA. We highlight the key aspects of CCA aetiopathogenesis and clinical management and provide insights into promising new treatments. Finally, we provide a set of consensus recommendations and future research priorities for CCA based on a Delphi panel questionnaire involving international experts.
Disciplines :
Surgery Oncology Gastroenterology & hepatology
Author, co-author :
Banales, Jesus M ; Department of Liver and Gastrointestinal Diseases, Biogipuzkoa Health Research Institute - Donostia University Hospital, University of the Basque Country (UPV/EHU), San Sebastian, Spain. jesusmaria.banalesasurmendi@bio-gipuzkoa.eus ; National Institute for the Study of Liver and Gastrointestinal Diseases (CIBERehd), Carlos III National Health Institute, Madrid, Spain. jesusmaria.banalesasurmendi@bio-gipuzkoa.eus ; Ikerbasque, Basque Foundation for Science, Bilbao, Spain. jesusmaria.banalesasurmendi@bio-gipuzkoa.eus ; Department of Biochemistry and Genetics, School of Sciences, University of Navarra, Pamplona, Spain. jesusmaria.banalesasurmendi@bio-gipuzkoa.eus
Rodrigues, Pedro M; Department of Liver and Gastrointestinal Diseases, Biogipuzkoa Health Research Institute - Donostia University Hospital, University of the Basque Country (UPV/EHU), San Sebastian, Spain. pedromiguel.rodriguesvieira@bio-gipuzkoa.eus ; National Institute for the Study of Liver and Gastrointestinal Diseases (CIBERehd), Carlos III National Health Institute, Madrid, Spain. pedromiguel.rodriguesvieira@bio-gipuzkoa.eus ; Ikerbasque, Basque Foundation for Science, Bilbao, Spain. pedromiguel.rodriguesvieira@bio-gipuzkoa.eus
Affò, Silvia ; Cancer Program, Tumor Microenvironment Plasticity and Heterogeneity Research Group, Institut d'Investigacions Biomèdiques August Pi i Sunyer, Barcelona, Spain
Andersen, Jesper B ; Biotech Research and Innovation Centre (BRIC), Department of Health and Medical Sciences, University of Copenhagen, Copenhagen, Denmark
Aspichueta, Patricia ; National Institute for the Study of Liver and Gastrointestinal Diseases (CIBERehd), Carlos III National Health Institute, Madrid, Spain ; Department of Physiology, Faculty of Medicine and Nursing, University of the Basque Country UPV/EHU, Leioa, Spain ; BioBizkaia Health Research Institute, Barakaldo, Spain
Boulter, Luke ; MRC Human Genetics Unit, Institute of Genetics and Cancer, Edinburgh, UK ; Cancer Research UK Scotland Centre, Edinburgh, UK
Bridgewater, John ; UCL Cancer Institute, Department of Medical Oncology, London, UK
Calvisi, Diego F ; Institute of Pathology, University of Regensburg, Regensburg, Germany
Cardenas, Andres; National Institute for the Study of Liver and Gastrointestinal Diseases (CIBERehd), Carlos III National Health Institute, Madrid, Spain ; GI and Liver Unit, Institut de Malalties Digestives, Hospital Clinic, Barcelona, Spain ; Institut d'Investigacions Biomèdiques August Pi-Sunyer (IDIBAPS), Barcelona, Spain
Cardinale, Vincenzo; Department of Translational and Precision Medicine, Sapienza University of Rome, Rome, Italy
Carpino, Guido ; Department of Anatomical, Histological, Forensic Medicine and Orthopaedic Sciences, Sapienza University of Rome, Rome, Italy
Coulouarn, Cédric ; Inserm, Univ Rennes, OSS (Oncogenesis, Stress, Signaling) UMR_S 1242, Centre de Lutte contre le Cancer Eugène Marquis, Rennes, France
Dopazo, Cristina; Department of HPB Surgery and Transplants, Vall d'Hebron Hospital Universitari, Vall d'Hebron Institut de Recerca (VHIR), Vall d'Hebron Barcelona Hospital Campus, Universitat Autónoma de Barcelona, Barcelona, Spain
Edeline, Julien ; Medical Oncology Department, Centre Eugene Marquis, Rennes, France
Fabris, Luca ; Department of Medicine, School of Medicine, University-Hospital of Padua, Padua, Italy ; Department of Internal Medicine, Digestive Disease Section, Yale University, New Haven, CT, USA
Folseraas, Trine; Norwegian PSC Research Center and Department of Transplantation Medicine, Division of Surgery, Inflammatory Diseases and Transplantation, Oslo University Hospital Rikshospitalet and University of Oslo, Oslo, Norway
Forner, Alejandro ; National Institute for the Study of Liver and Gastrointestinal Diseases (CIBERehd), Carlos III National Health Institute, Madrid, Spain ; Liver Oncology Unit (BCLC group), Liver Unit, ICMDM, Hospital Clinic of Barcelona, University of Barcelona, Barcelona, Spain
Goeppert, Benjamin ; Institute of Pathology, RKH Klinikum, Ludwigsburg, Germany ; Institute of Tissue Medicine and Pathology (ITMP), University Bern, Bern, Switzerland
Heikenwalder, Mathias; Division of Chronic Inflammation and Cancer, German Cancer Research Center (DKFZ), Heidelberg, Germany ; M3 Research Center for Malignome, Metabolome and Microbiome, Institute for Interdisciplinary Research on Cancer Metabolism and Chronic Inflammation, University of Tübingen, Tübingen, Germany
Kendall, Timothy J ; Centre for Inflammation Research, Institute for Regeneration and Repair, University of Edinburgh, Edinburgh, UK
Khan, Shahid A ; Department of Hepatology, Imperial College Healthcare NHS Trust, London, UK
Klümpen, Heinz-Josef ; Department of Medical Oncology, Amsterdam UMC Location University of Amsterdam, Amsterdam, The Netherlands
Koerkamp, Bas Groot ; Erasmus MC, Department of Surgery, Rotterdam, The Netherlands
Lamarca, Angela; Department of Oncology - OncoHealth Institute - Instituto de Investigaciones Sanitarias FJD, Fundación Jiménez Díaz University Hospital, Madrid, Spain
Lindsey, Stacie; Cholangiocarcinoma Foundation, Salt Lake City, UT, USA
Lleo, Ana ; Department of Biomedical Sciences, Humanitas University, Pieve Emanuele, Milan, Italy ; Division of Internal Medicine and Hepatology, Department of Gastroenterology, IRCCS Humanitas Research Hospital, Rozzano, Milan, Italy
Luedde, Tom ; Department of Gastroenterology, Hepatology and Infectious Diseases, University Hospital Duesseldorf, Medical Faculty of Heinrich Heine University Duesseldorf, Duesseldorf, Germany
Macias, Rocio I R ; National Institute for the Study of Liver and Gastrointestinal Diseases (CIBERehd), Carlos III National Health Institute, Madrid, Spain ; Experimental Hepatology and Drug Targeting (HEVEPHARM), Institute for Biomedical Research of Salamanca (IBSAL), University of Salamanca, Salamanca, Spain
Morement, Helen ; AMMF, The Cholangiocarcinoma Charity, Essex, UK
Nault, Jean-Charles; Liver Unit, Avicenne Hospital, APHP, Paris Nord University, Bobigny, France ; Cordeliers Research Center, Sorbonne Université, Inserm, Université de Paris, Team «Functional Genomics of Solid Tumours», Equipe labellisée Ligue Nationale Contre le Cancer, Labex OncoImmunology, Paris, France
Olaizola, Paula; National Institute for the Study of Liver and Gastrointestinal Diseases (CIBERehd), Carlos III National Health Institute, Madrid, Spain ; MRC Human Genetics Unit, Institute of Genetics and Cancer, Edinburgh, UK
Perugorria, Maria J; Department of Liver and Gastrointestinal Diseases, Biogipuzkoa Health Research Institute - Donostia University Hospital, University of the Basque Country (UPV/EHU), San Sebastian, Spain ; National Institute for the Study of Liver and Gastrointestinal Diseases (CIBERehd), Carlos III National Health Institute, Madrid, Spain ; Department of Medicine, Faculty of Medicine and Nursing, University of the Basque Country (UPV/EHU), San Sebastian, Spain
Raggi, Chiara ; Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy
Rimassa, Lorenza ; Department of Biomedical Sciences, Humanitas University, Pieve Emanuele, Milan, Italy ; Medical Oncology and Hematology Unit, Humanitas Cancer Center, IRCCS Humanitas Research Hospital, Rozzano, Milan, Italy
Saborowski, Anna ; Department of Gastroenterology, Hepatology, Infectiology and Endocrinology, Hannover Medical School, Hannover, Germany
Valle, Juan W ; Cholangiocarcinoma Foundation, Salt Lake City, UT, USA ; Division of Cancer Sciences, University of Manchester, Manchester, UK
Vithayathil, Mathew ; Department of Surgery and Cancer, Imperial College London, London, UK
Vogel, Arndt ; Department of Gastroenterology, Hepatology, Infectiology and Endocrinology, Hannover Medical School, Hannover, Germany ; Division of Gastroenterology and Hepatology, Toronto General Hospital, Toronto, Ontario, Canada ; Medical Oncology, Princess Margaret Cancer Centre, Toronto, Ontario, Canada
Braconi, Chiara ; School of Cancer Sciences, University of Glasgow, Glasgow, UK ; CRUK Scotland Cancer Centre, Glasgow, UK ; Beatson West of Scotland Cancer Centre, Glasgow, UK
Detry, Olivier ; Université de Liège - ULiège > Département des sciences cliniques > Pathologie chirurgicale abdominale et endocrinienne ; Centre Hospitalier Universitaire de Liège - CHU > > Service de chirurgie abdo, sénologique, endocrine et de transplantation
Troisfontaine, Florence ; Université de Liège - ULiège > Département des sciences cliniques ; Centre Hospitalier Universitaire de Liège - CHU > > Service d'oncologie médicale
Language :
English
Title :
Cholangiocarcinoma 2026: status quo, unmet needs and priorities.