Cancer risk in primary sclerosing cholangitis: Epidemiology, prevention, and surveillance strategies

Brian M. Fung, Keith Lindor, James H. Tabibian

Research output: Contribution to journalReview article

Abstract

Primary sclerosing cholangitis (PSC) is a rare cholestatic liver disease characterized by progressive fibroinflammatory destruction of the intra- and/or extrahepatic biliary ducts. While its features and disease course can be variable, most patients with PSC have concurrent inflammatory bowel disease and will eventually develop liver cirrhosis and end-stage liver disease, with liver transplantation representing the only potentially curative option. Importantly, PSC is associated with a significantly increased risk of malignancy compared to the general population, mainly cholangiocarcinoma, gallbladder carcinoma, hepatocellular carcinoma, and colorectal cancer, with nearly 50% of deaths in patients with PSC being due to cancer. Therefore, robust surveillance strategies are needed, though uncertainty remains regarding how to best do so. In this review, we discuss the epidemiology, prevention, and surveillance of cancers in patients with PSC. Where evidence is limited, we present pragmatic approaches based on currently available data and expert opinion.

Original languageEnglish (US)
Pages (from-to)659-671
Number of pages13
JournalWorld journal of gastroenterology
Volume25
Issue number6
DOIs
StatePublished - Feb 14 2019

Fingerprint

Sclerosing Cholangitis
Epidemiology
Neoplasms
End Stage Liver Disease
Cholangiocarcinoma
Expert Testimony
Liver Neoplasms
Gallbladder
Inflammatory Bowel Diseases
Liver Cirrhosis
Liver Transplantation
Uncertainty
Liver Diseases
Hepatocellular Carcinoma
Colorectal Neoplasms
Carcinoma
Population

Keywords

  • Bile duct diseases
  • Chemoprotection
  • Cholangiocarcinoma
  • Colorectal cancer
  • Gallbladder carcinoma
  • Hepatocellular carcinoma
  • Inflammatory bowel disease

ASJC Scopus subject areas

  • Gastroenterology

Cite this

Cancer risk in primary sclerosing cholangitis : Epidemiology, prevention, and surveillance strategies. / Fung, Brian M.; Lindor, Keith; Tabibian, James H.

In: World journal of gastroenterology, Vol. 25, No. 6, 14.02.2019, p. 659-671.

Research output: Contribution to journalReview article

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