Cholangiocarcinoma complicating primary sclerosing cholangitis: cholangiographic appearances.

Cholangiograms from 104 patients (and serial cholangiograms in 66 patients) with primary sclerosing cholangitis (PSC) were reviewed. In 13 patients the additional diagnosis of cholangiocarcinoma was made at biopsy or autopsy. Cholangiograms from patients with both PSC and carcinoma were compared with cholangiograms from patients with PSC alone. Marked dilatation of ducts or ductal segments (100% vs. 24%) and the appearance of a polypoid mass (46% vs. 7%) were common findings in the group of patients whose disease was complicated by malignancy. In the malignant group, polypoid masses were larger, measuring 1 cm or greater in diameter. On serial cholangiograms, four of 15 patients with progressive stricture formation and four of five with progressive ductal dilatation proved to have carcinomas. The frequent occurrence of bile duct carcinoma as a complication of PSC in this group of patients indicates that PSC has a strong tendency to undergo malignant degeneration. Cholangiographic findings which suggest malignant degeneration include markedly dilated ducts or ductal segments, presence of a polypoid mass 1 cm or greater in diameter, and progressive stricture formation or ductal dilatation.