Incidence of Hepatobiliary Malignancies in Primary Sclerosing Cholangitis: Systematic Review and Meta-analysis

Primary sclerosing cholangitis (PSC) is a known risk factor for hepatobiliary malignancies. We conducted a systematic review and meta-analysis of published studies to determine the incidence and risk factors for hepatobiliary malignancies in people with PSC.

Pubmed and Embase databases were searched from inception to April 10, 2024, for cohort studies reporting data on the incidence of cholangiocarcinoma (CCA), hepatocellular carcinoma (HCC), or gallbladder cancer (GBC) in PSC. Pooled incidence rates with 95% confidence intervals (CIs) were estimated using a random effects model.

We identified 51 eligible studies involving 26,482 patients. The total follow-up was 221,258.1 person-years (PYs). The pooled incidence rates for overall PSC were 9.31 (95% CI, 6.84-12.67; I² = 74%), 1.73 (95% CI, 1.20-2.51; I² = 55%), and 1.06 (95% CI, 0.85-1.31; I² = 0%) per 1000 PYs for CCA, HCC, and GBC, respectively. In patients with PSC with inflammatory bowel disease (IBD), rates were 7.16 (95% CI, 4.48-11.44; I² = 96%), 2.19 (95% CI, 1.48-3.25; I² = 58%), and 1.52 (95% CI, 1.21-1.90; I² = 0%) per 1000 PYs, respectively. Subgroup analysis showed that the incidence of CCA was higher in smaller studies (<200 patients), and the incidence of HCC varied significantly by region (P = .03), with Oceania having the highest incidence and Europe having the lowest. Meta-regression determined that PSC-IBD was associated with HCC incidence.

The incidence of CCA in PSC is substantial, whereas HCC and GBC are rare. Patients with PSC-IBD may be at higher risk for HCC. These data should be validated in large, prospective studies, and may guide the development of evidence-based surveillance strategies for hepatobiliary malignancies in PSC.