Cholangiocarcinoma (CCA) is a heterogeneous group of dysplastic disorders affecting
the biliary epithelium. It is the second most common primary liver tumor which accounts for around
3% of all gastrointestinal cancers. CCA is very deadly due to its aggressiveness, late diagnosis and
high chemoresistance. The incidence is increasing worldwide and the therapeutic options are very
limited. Radiotherapy, chemotherapy, surgery and/or liver transplantation may be indicated in patients
who meet certain criteria, but chances of success are low. There is therefore increasing interest
in understanding the molecular mechanisms involved in the pathogenesis of this cancer type and in
identifying new targets for therapy. Current strategies are based on targeting key signaling pathways
involved in proliferation, survival, apoptosis and migration. In this review, the most relevant molecular
mechanisms involved in the pathogenesis of CCA are discussed and the main preclinical and
clinical studies are highlighted. Moreover, future directions in basic and clinical research are indicated.
Keywords: Biliary epithelium, cholangiocarcinoma, cancer, pathogenesis, stroma, molecular mechanisms, therapy.
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