Brindley, Paul J., Bachini, Melinda, Ilyas, Sumera I., Khan, Shahid A., Loukas, Alex, Sirica, Alphonse E., Teh, Bin Tean, Wongkham, Sopit, and Gores, Gregory J. (2021) Cholangiocarcinoma. Nature Reviews Disease Primers, 7. 65. pp. 1-17.

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Cholangiocarcinoma (CCA) is a highly lethal adenocarcinoma of the hepatobiliary system, which can be classified as intrahepatic, perihilar and distal. Each anatomic subtype has distinct genetic aberrations, clinical presentations and therapeutic approaches. In endemic regions, liver fluke infection is associated with CCA, owing to the oncogenic effect of the associated chronic biliary tract inflammation. In other regions, CCA can be associated with chronic biliary tract inflammation owing to choledocholithiasis, cholelithiasis, or primary sclerosing cholangitis, but most CCAs have no identifiable cause. Administration of the anthelmintic drug praziquantel decreases the risk of CCA from liver flukes, but reinfection is common and future vaccination strategies may be more effective. Some patients with CCA are eligible for potentially curative surgical options, such as resection or liver transplantation. Genetic studies have provided new insights into the pathogenesis of CCA, and two aberrations that drive the pathogenesis of non-fluke-associated intrahepatic CCA, fibroblast growth factor receptor 2 fusions and isocitrate dehydrogenase gain-of-function mutations, can be therapeutically targeted. CCA is a highly desmoplastic cancer and targeting the tumour immune microenvironment might be a promising therapeutic approach. CCA remains a highly lethal disease and further scientific and clinical insights are needed to improve patient outcomes.

Item ID: 69938
Item Type: Article (Research - C1)
ISSN: 2056-676X
Copyright Information: © Springer Nature Limited 2021.
Date Deposited: 26 May 2022 01:38
FoR Codes: 42 HEALTH SCIENCES > 4202 Epidemiology > 420207 Major global burdens of disease @ 100%
SEO Codes: 20 HEALTH > 2001 Clinical health > 200105 Treatment of human diseases and conditions @ 100%
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