Liver transplantation in primary sclerosing cholangitis with hepatobiliary neoplasia: survival outcomes and impact of diagnostic uncertainty

Background and aims Primary sclerosing cholangitis (PSC) is associated with an increased risk of hepatobiliary malignancy, with cholangiocarcinoma (CCA) representing a leading cause of death. We aimed to evaluate characteristics and outcome after liver transplantation (LT) in individuals with PSC who had biliary high-grade dysplasia (HGD), CCA, hepatocellular carcinoma (HCC) or gallbladder carcinoma (GBC) in the liver explants.Method A retrospective cohort study was conducted on individuals with PSC who underwent LT and had HGD, CCA, GBC and HCC identified by explant histology. Individuals were identified through the Nordic Liver Transplant Registry, and the original explant histology was assessed. One hundred (n = 100) individuals with PSC were included, all aged ≥18 years and liver transplanted between 2000 and 2021 at Oslo and Karolinska University Hospitals. Clinicopathological data and outcome were assessed, and survival analyses performed.Results Out of all transplanted individuals with PSC and primary hepatobiliary neoplasms (n = 100), HGD was diagnosed in 33% (n = 33), CCA in 45% (n = 45), HCC in 17% (n = 17) and GBC in 5% (n = 5) of explants. The 1-, 3- and 5-year survivals for HGD were 97%, 91% and 87%, respectively. Five-year survival was higher for individuals with HCC (82%) and HGD (87%) than for those with CCA (32%). Recurrence of hepatobiliary malignancy was less frequently observed in individuals with HCC than in those with CCA (18% vs. 58%, p = 0.004).Conclusion LT in individuals with PSC-HGD and PSC-HCC, including both preoperatively suspected and incidental cases, is associated with good long-term outcomes. In contrast, the poor survival among transplanted individuals with PSC-CCA underscores the need of earlier cancer detection and optimized oncological therapy.