Primary sclerosing cholangitis complicated with cholangiocarcinoma

Abstract

Background: Primary sclerosing cholangitis (PSC) complicated with cholangiocarcinoma (CC) is rarely reported in Japan. Methods: In this study, we investigated 38 patients with PSC retrospectively. A total of 5 patients (13.2%) had CC. This rate corresponds to that reported in Western countries. Results: The mean age at CC diagnosis was 49.6 years (range: 31-65 years), and 2 patients were women. Three of 5 patients did not suffer from cirrhosis. No significant difference was observed in background characteristics between patients with and without CC. Three of 5 patients were diagnosed with CC at the same time or within the first year after diagnosis of PSC. CC manifested as bile duct stricture or mass formation. Two of 5 patients exhibited mass formation in this study. The prognosis of patients with CC was poor, and all patients died. The median survival time was 1.04 years (range: 0.2-3.3 years). Conclusions: Patients with PSC should be carefully monitored for CC development using an imaging modality, particularly within the first year after diagnosis of PSC.