Cholangiocarcinoma.
Journal
Nature reviews. Disease primers
ISSN: 2056-676X
Titre abrégé: Nat Rev Dis Primers
Pays: England
ID NLM: 101672103
Informations de publication
Date de publication:
09 09 2021
09 09 2021
Historique:
accepted:
03
08
2021
entrez:
10
9
2021
pubmed:
11
9
2021
medline:
26
11
2021
Statut:
epublish
Résumé
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.
Identifiants
pubmed: 34504109
doi: 10.1038/s41572-021-00300-2
pii: 10.1038/s41572-021-00300-2
pmc: PMC9246479
mid: NIHMS1816060
doi:
Types de publication
Journal Article
Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
65Subventions
Organisme : NCI NIH HHS
ID : K08 CA236874
Pays : United States
Organisme : NIAID NIH HHS
ID : P50 AI098639
Pays : United States
Organisme : NCI NIH HHS
ID : P50 CA210964
Pays : United States
Organisme : NCI NIH HHS
ID : R01 CA164719
Pays : United States
Informations de copyright
© 2021. Springer Nature Limited.
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