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Cholangiocarcinoma: Disease Pathogenesis and New Treatment Paradigms

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Biliary Disease

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Abstract

Cholangiocarcinoma (CCA) is a malignancy arising in the biliary tree. Its incidence rates have increased, and it has surpassed gallbladder carcinoma as the most common biliary tract malignancy. Most patients are diagnosed with advanced stage disease and are not amenable to potentially curative surgical therapies. The prognosis is dismal with a median survival of <24 months. Novel possible epidemiologic risk factors (i.e., diabetes, cirrhosis, metabolic syndrome) have been identified. Based upon clinical behavior and distinct molecular patterns, intrahepatic, perihilar, and distal CCAs are characterized as distinct malignancies that need to be treated as such in their management and clinical trials. While cholestasis and inflammation are key factors for all three CCA subtypes, their genetic profile differs. Because knowledge of the genetic landscape of CCA has resulted in multiple new therapeutic strategies and clinical trials, it will become increasingly possible to develop precision medicine-based therapies for CCA patients.

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Author information

Authors and Affiliations

  1. Mayo Clinic, Rochester, MN, USA

    Gregory J. Gores MD

  2. MD Anderson Cancer Center, Houston, TX, USA

    Boris Blechacz MD, PhD

Authors
  1. Gregory J. Gores MD
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  2. Boris Blechacz MD, PhD
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Corresponding author

Correspondence to Gregory J. Gores MD .

Editor information

Editors and Affiliations

  1. Centre for Liver Research NIHR Biomedical Research Unit, Institute of Immunology and Immunotherapy University of Birmingham, Birmingham, United Kingdom

    Gideon Hirschfield

  2. Centre for Liver Research NIHR Biomedical Research Unit, Institute of Immunology and Immunotherapy University of Birmingham, Birmingham, United Kingdom

    David Adams

  3. Centre for Liver Research NIHR Biomedical Research Unit, Institute of Immunology and Immunotherapy University of Birmingham, Birmingham, United Kingdom

    Evaggelia Liaskou

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Gores, G.J., Blechacz, B. (2017). Cholangiocarcinoma: Disease Pathogenesis and New Treatment Paradigms. In: Hirschfield, G., Adams, D., Liaskou, E. (eds) Biliary Disease. Springer, Cham. https://doi.org/10.1007/978-3-319-50168-0_10

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  • DOI: https://doi.org/10.1007/978-3-319-50168-0_10

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-50166-6

  • Online ISBN: 978-3-319-50168-0

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