Expert consensus document: Cholangiocarcinoma: current knowledge and future perspectives consensus statement from the European Network for the Study of Cholangiocarcinoma (ENS-CCA)

Nat Rev Gastroenterol Hepatol. 2016 May;13(5):261-80. doi: 10.1038/nrgastro.2016.51. Epub 2016 Apr 20.


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: or 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.

Publication types

  • Consensus Development Conference

MeSH terms

  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Bile Duct Neoplasms / genetics
  • Bile Duct Neoplasms / pathology*
  • Bile Duct Neoplasms / therapy
  • Cancer-Associated Fibroblasts / physiology
  • Cell Proliferation / physiology
  • Cell Transformation, Neoplastic / genetics
  • Cholangiocarcinoma / genetics
  • Cholangiocarcinoma / pathology*
  • Cholangiocarcinoma / therapy
  • Cytokines / metabolism
  • Drug Resistance, Neoplasm / genetics
  • Early Detection of Cancer
  • Epigenesis, Genetic / genetics
  • Forecasting
  • Gene Fusion / genetics
  • Genetic Heterogeneity
  • Humans
  • Intercellular Signaling Peptides and Proteins / metabolism
  • Liver Transplantation
  • Macrophages / physiology
  • Molecular Targeted Therapy / methods
  • Neoplasm Proteins / physiology
  • Neoplasm Staging
  • Neoplastic Stem Cells / pathology
  • Receptor, Fibroblast Growth Factor, Type 2 / genetics
  • Risk Factors
  • Signal Transduction / genetics
  • Stents
  • Tumor Microenvironment / genetics


  • Cytokines
  • Intercellular Signaling Peptides and Proteins
  • Neoplasm Proteins
  • FGFR2 protein, human
  • Receptor, Fibroblast Growth Factor, Type 2