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Cholangiocarcinoma: Epidemiology, Pathogenesis, and Prognosis

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Hepato-Pancreato-Biliary Malignancies

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Abstract

Cholangiocarcinoma is a malignancy arising from epithelial cells of the biliary tract characterized by its aggressive natural history, late diagnosis, and often fatal outcome. It is the most common biliary malignancy accounting for approximately 15–20% of all primary hepatobiliary cancers. Cholangiocarcinoma is classically subdivided based on anatomic location: intrahepatic, hilar, and distal. Each subtype is distinct in epidemiologic trend, natural history of progression, and prognosis. There are substantial geographical variations in incidence with the highest rates reported in Southeast Asia. Cholangiocarcinogenesis is driven by a complex multistep process beginning with chronic biliary tract inflammation followed by exposure to carcinogenic molecules and overexpression of proliferative growth factors. This results in stepwise accumulation of genetic mutations, ultimately leading to uncontrolled cell growth and metastasis. Patients with non-resectable advanced-stage cholangiocarcinoma have a dismal prognosis with a median survival of less than 24 months after diagnosis. If patients are diagnosed at an early stage, complete tumor resection is the only treatment that offers the best chance of cure and long-term survival. Unfortunately, despite R0 negative margin resections, long-term prognosis defined by survival and tumor recurrence remains poor in the presence of aggressive tumor-related factors.

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Authors and Affiliations

  1. Stony Brook Medicine, New York, NY, USA

    Yunseok Namn & Juan Carlos Bucobo

Authors
  1. Yunseok Namn
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  2. Juan Carlos Bucobo
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Correspondence to Juan Carlos Bucobo .

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Editors and Affiliations

  1. Medical Director, Capital Health Cancer Center, Director, CH Pancreas Center of Excellence, Director, CH Liver Center of Excellence, Pennington, NJ, USA

    Cataldo Doria

  2. Director of Interventional Gastroenterology, Capital Health Medical Center, Pennington, NJ, USA

    Jason N. Rogart

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Namn, Y., Bucobo, J.C. (2022). Cholangiocarcinoma: Epidemiology, Pathogenesis, and Prognosis. In: Doria, C., Rogart, J.N. (eds) Hepato-Pancreato-Biliary Malignancies. Springer, Cham. https://doi.org/10.1007/978-3-030-41683-6_13

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  • DOI: https://doi.org/10.1007/978-3-030-41683-6_13

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-41682-9

  • Online ISBN: 978-3-030-41683-6

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