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Determining the time to cholangiocarcinoma in pediatric-onset PSC-IBD
Batul Kaj-Carbaidwala1, Johan Fevery2, Douglas G Adler3
1Division of Pediatric Gastroenterology, Hepatology and Nutrition, MassGeneral Hospital for Children, Boston, Massachusetts, USA.
Insights
Primary sclerosing cholangitis (PSC) and inflammatory bowel disease (IBD) in children increase cholangiocarcinoma risk. Early screening is crucial, especially during the pediatric to adult care transition period.
Area of Science:
- Pediatric Gastroenterology
- Hepatology
- Oncology
Background:
- Primary sclerosing cholangitis (PSC) is a known risk factor for cholangiocarcinoma.
- Limited evidence exists for managing cholangiocarcinoma risk in pediatric patients with both PSC and inflammatory bowel disease (IBD).
Purpose of the Study:
- To determine the time interval between diagnosis of PSC-IBD and the development of cholangiocarcinoma in pediatric patients.
- To inform family counseling and risk management strategies for this patient population.
Main Methods:
- A case series was conducted using data from PubMed and collaborators.
- Included patients with pediatric-onset PSC-IBD who subsequently developed cholangiocarcinoma.
- Authors were contacted to establish an event curve for cholangiocarcinoma development.
Main Results:
- A cohort of 21 patients with pediatric-onset PSC-IBD-cholangiocarcinoma was identified from 175 studies.
- The median time to cholangiocarcinoma diagnosis was 6.95 years after the second diagnosis (PSC or IBD).
- Notably, 38% of cholangiocarcinomas occurred within 2 years, and 47% developed during the transition to adult care (ages 14-25).
Conclusions:
- Pediatric patients with PSC-IBD face a significant risk of developing cholangiocarcinoma.
- Screening for cholangiocarcinoma should extend beyond pediatric care into the transition period to adult care.
- Early and ongoing surveillance is critical for early detection and management.
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.
