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Review
. 2025 Mar;80(3):450-454.
doi: 10.1002/jpn3.12443. Epub 2024 Dec 20.

Determining the time to cholangiocarcinoma in pediatric-onset PSC-IBD

Batul Kaj-Carbaidwala  1 , Johan Fevery  2 , Douglas G Adler  3 , Annika Bergquist  4 , Lissy de Ridder  5 , Mark Deneau  6 , Corinne Gower-Rousseau  7 , Roger W Chapman  8 , Kate D Lynch  8   9 , Catherine A M Stedman  10 , David C Wilson  11 , Uzma Shah  1 , Lipika Goyal  12 , Harland S Winter  1 , Jochen K Lennerz  13
Affiliations
Review

Determining the time to cholangiocarcinoma in pediatric-onset PSC-IBD

Batul Kaj-Carbaidwala et al. J Pediatr Gastroenterol Nutr. 2025 Mar.

Abstract

Primary sclerosing cholangitis (PSC) is a risk factor for cholangiocarcinoma. When a child is diagnosed with both PSC and inflammatory bowel disease (IBD), evidence-based information on counseling families and risk management of developing cholangiocarcinoma is limited. In this case series (PubMed/collaborators), we included patients with PSC-IBD who developed cholangiocarcinoma and contacted authors to determine an event curve specifying the time between the second diagnosis (IBD or PSC) and a diagnosis of cholangiocarcinoma. Review of n = 175 studies resulted in a cohort of n = 21 patients with pediatric-onset PSC-IBD-cholangiocarcinoma. The median time to development of cholangiocarcinoma was 6.95 years from the second diagnosis. Despite the small number, 38% of cholangiocarcinoma developed within the first 2 years, and 47% of patients developed cholangiocarcinoma in the transition period to adult care (age 14-25). Our findings highlight the importance of screening that extends over the so-called transition period from pediatric to adult care.

Keywords: Crohn's; pediatric cholangitis; primary sclerosing cholangitis; ulcerative colitis.

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References

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