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 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.