Segmental primary sclerosing cholangitis mimicking bile duct cancer--report of a case and review of the Japanese literature.

A rare case of the segmental type of primary sclerosing cholangitis (PSC) is reported herein. A 27 year old Japanese man with obstructive jaundice was referred to our hospital with a provisional diagnosis of bile duct cancer at the hepatic hilum. A PTCD tube had been inserted from the right anterior segmental duct and direct cholangiograms showed complete obstruction of the bile duct at the confluence of the right and left hepatic ducts. Resection of the extrahepatic bile duct and partial hepatectomy were thus performed, however, the postoperative histological examination revealed no malignant features. Instead, the lesion was shown to be compatible with PSC microscopically. A diagnosis of PSC was finally established on the basis of clinical features, cholangiographic appearance and the pathological report.