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Cholangiocarcinoma

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Diseases of the Liver and Biliary Tree

Abstract

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.

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Authors and Affiliations

  1. General Medicine Division, Azienda Ospedale-Università di Padova, Padova, Italy

    Alberto Lasagni & Luca Fabris

  2. Digestive Disease Section, Yale University, New Haven, CT, USA

    Mario Strazzabosco & Luca Fabris

  3. Department of Pathology, Treviso Regional Hospital, Azienda ULSS2 Marca Trevigiana, Treviso, Italy

    Maria Guido

  4. Department of Medicine-DIMED, University of Padova, Padova, Italy

    Maria Guido

  5. Department of Molecular Medicine-DMM, University of Padova, Padova, Italy

    Luca Fabris & Massimiliano Cadamuro

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  1. Scientific Consultant, Scientific Institute for Research, Hospitalization and Healthcare (IRCCS) Negrar, Verona, Italy

    Annarosa Floreani

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Lasagni, A., Strazzabosco, M., Guido, M., Fabris, L., Cadamuro, M. (2021). Cholangiocarcinoma. In: Floreani, A. (eds) Diseases of the Liver and Biliary Tree. Springer, Cham. https://doi.org/10.1007/978-3-030-65908-0_14

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