Expert consensus document: Cholangiocarcinoma: current knowledge and future perspectives consensus statement from the European Network for the Study of Cholangiocarcinoma (ENS-CCA).
Cholangiocarcinoma (CCA) is a heterogeneous group of malignancies with features of biliary tract differentiation. CCA is the second most common primary liver tumour and the incidence is increasing worldwide. CCA has high mortality owing to its aggressiveness, late diagnosis and refractory nature. In May 2015, the "European Network for the Study of Cholangiocarcinoma" (ENS-CCA: www.enscca.org or www.cholangiocarcinoma.eu) was created to promote and boost international research collaboration on the study of CCA at basic, translational and clinical level. In this Consensus Statement, we aim to provide valuable information on classifications, pathological features, risk factors, cells of origin, genetic and epigenetic modifications and current therapies available for this cancer. Moreover, future directions on basic and clinical investigations and plans for the ENS-CCA are highlighted.
Department of Liver and Gastrointestinal Diseases, Biodonostia Health Research Institute - Donostia University Hospital, Ikerbasque, CIBERehd, Paseo del Dr. Begiristain s/n, E-20014, San Sebastian, Spain.
Jesus M
JM
Banales
Department of Medico-Surgical Sciences and Biotechnologies, Sapienza University of Rome, Viale dell'Università 37, 00185, Rome, Italy.
Vincenzo
V
Cardinale
Department of Movement, Human and Health Sciences, University of Rome "Foro Italico", Piazza Lauro De Bosis 6, 00135, Rome, Italy.
Guido
G
Carpino
Department of Clinic and Molecular Sciences, Polytechnic University of Marche, Via Tronto 10, 60020, Ancona, Italy.
Marco
M
Marzioni
Biotech Research and Innovation Centre, University of Copenhagen, Ole Maaløes Vej 5, DK-2200, Copenhagen N, Denmark.
Jesper B
JB
Andersen
Humanitas Clinical and Research Center, Via Manzoni 56, Rozzano, 20089, Milan, Italy.|Program for Autoimmune Liver Diseases, International Center for Digestive Health, Department of Medicine and Surgery, University of Milan-Bicocca, Via Cadore 48, 20900, Monza, Italy.
Pietro
P
Invernizzi
Department of Molecular Oncology, Institute for Cancer Research, Oslo University Hospital, The Norwegian Radium Hospital, Montebello, 0310, Oslo, Norway.
Guro E
GE
Lind
Department of Transplantation Medicine, Division of Cancer Medicine, Surgery and Transplantation, Oslo University Hospital, Rikshospitalet, Pb. 4950 Nydalen, N-0424, Oslo, Norway.
Trine
T
Folseraas
MRC Centre for Regenerative Medicine, University of Edinburgh, 49 Little France Crescent, EH16 4SB, Edinburgh, United Kingdom.
Stuart J
SJ
Forbes
INSERM UMR S938, Centre de Recherche Saint-Antoine, 184 rue du Faubourg Saint-Antoine, 75571, Paris cedex 12, Fondation ARC, 9 rue Guy Môquet 94803 Villejuif, France.
Laura
L
Fouassier
Department of Internal Medicine II, University Hospital Würzburg, Oberdürrbacherstrasse 6, D-97080, Würzburg, Germany.
Andreas
A
Geier
Institute of Pathology, Universitätsmedizin Greifswald, Friedrich-Löffler-Strasse 23e, 17489, Greifswald, Germany.
Diego F
DF
Calvisi
Division of Gastroenterology and Hepatology, University Hospital Zurich, Rämistrasse 100, 8091, Zürich, Switzerland.
Joachim C
JC
Mertens
Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Waehringer Guertel 18-20, A-1090, Vienna, Austria.
Michael
M
Trauner
Department of Clinic and Molecular Sciences, Polytechnic University of Marche, Via Tronto 10, 60020, Ancona, Italy.
Antonio
A
Benedetti
Department of Clinic and Molecular Sciences, Polytechnic University of Marche, Via Tronto 10, 60020, Ancona, Italy.
Luca
L
Maroni
INSERM UMR S938, Centre de Recherche Saint-Antoine, 184 rue du Faubourg Saint-Antoine, 75571, Paris cedex 12, Fondation ARC, 9 rue Guy Môquet 94803 Villejuif, France.
Javier
J
Vaquero
Department of Physiology and Pharmacology, Experimental Hepatology and Drug Targeting (HEVEFARM), Campus Miguel de Unamuno, E.I.D. S-09, University of Salamanca, IBSAL, CIBERehd, 37007, Salamanca, Spain.
Rocio I R
RI
Macias
Humanitas Clinical and Research Center, Via Manzoni 56, Rozzano, 20089, Milan, Italy.
Chiara
C
Raggi
Department of Liver and Gastrointestinal Diseases, Biodonostia Health Research Institute - Donostia University Hospital, Ikerbasque, CIBERehd, Paseo del Dr. Begiristain s/n, E-20014, San Sebastian, Spain.
Maria J
MJ
Perugorria
Department of Anatomical, Histological, Forensic Medicine and Orthopedics Sciences, Sapienza University of Rome, Via Alfonso Borelli 50, 00161, Rome, Italy.
Eugenio
E
Gaudio
Department of Transplantation Medicine, Division of Cancer Medicine, Surgery and Transplantation, Oslo University Hospital, Rikshospitalet, Pb. 4950 Nydalen, N-0424, Oslo, Norway.
Kirsten M
KM
Boberg
Department of Physiology and Pharmacology, Experimental Hepatology and Drug Targeting (HEVEFARM), Campus Miguel de Unamuno, E.I.D. S-09, University of Salamanca, IBSAL, CIBERehd, 37007, Salamanca, Spain.
Jose J G
JJ
Marin
Department of Medico-Surgical Sciences and Biotechnologies, Sapienza University of Rome, Viale dell'Università 37, 00185, Rome, Italy.
Domenico
D
Alvaro
D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001650:Bile Duct Neoplasms / Q000235:genetics / Q000473:pathology* / Q000628:therapy; D000072645:Cancer-Associated Fibroblasts / Q000502:physiology; D049109:Cell Proliferation / Q000502:physiology; D002471:Cell Transformation, Neoplastic / Q000235:genetics; D018281:Cholangiocarcinoma / Q000235:genetics / Q000473:pathology* / Q000628:therapy; D016207:Cytokines / Q000378:metabolism; D019008:Drug Resistance, Neoplasm / Q000235:genetics; D055088:Early Detection of Cancer; D044127:Epigenesis, Genetic / Q000235:genetics; D005544:Forecasting; D050939:Gene Fusion / Q000235:genetics; D018740:Genetic Heterogeneity; D006801:Humans; D036341:Intercellular Signaling Peptides and Proteins / Q000378:metabolism; D016031:Liver Transplantation; D008264:Macrophages / Q000502:physiology; D058990:Molecular Targeted Therapy / Q000379:methods; D009363:Neoplasm Proteins / Q000502:physiology; D009367:Neoplasm Staging; D014411:Neoplastic Stem Cells / Q000473:pathology; D051497:Receptor, Fibroblast Growth Factor, Type 2 / Q000235:genetics; D012307:Risk Factors; D015398:Signal Transduction / Q000235:genetics; D015607:Stents; D059016:Tumor Microenvironment / Q000235:genetics
D016207:Cytokines; D036341:Intercellular Signaling Peptides and Proteins; D009363:Neoplasm Proteins; C496353:FGFR2 protein, human; D051497:Receptor, Fibroblast Growth Factor, Type 2