Cholangiocarcinoma

Cholangiocarcinoma (CCA) includes a group of different epithelial cancers with features of biliary tract differentiation arising from any tract of the biliary tree. Histologically, they usually are adenocarcinomas. It is a rare cancer accounting about 3% of all gastrointestinal malignancies. Based on its anatomical location, CCA is classified as intrahepatic (iCCA), perihilar (pCCA), and distal (dCCA). Currently, the three types of CCA are considered as distinct cancers since different pathogenic and management features. Cholangiocarcinomas are aggressive tumours, often diagnosed at advanced stages. Most cases are sporadic but conditions leading to chronic inflammation and cholestasis have been recognized as risk factors. Diagnosis needs a multimodal approach, mixing imaging, endoscopy, laboratory tests, including onco-biomarkers, and pathology. Surgical resection with histologically negative margins is the only curative treatment, although it is possible only for few patients; unfortunately, recurrence is frequent. Likewise, liver transplantation is an option for a small subset of selected patients suffering from pCCA. Generally, prognosis is poor for most patients. Desmoplastic nature, highly variable genetics, interaction with a rich tumour microenvironment, all contribute to the resistance of therapy. Advances in targeted-, radio- and immunotherapy will lead to improvement in survival.