A localized primary sclerosing cholangitis preoperatively diagnosed as hilar cholangiocarcinoma; report of a case.

The authors report herein a case of primary sclerosing cholangitis localized to the hepatic hilum which occurred in a 67-year-old male. The direct cholangiography revealed bile duct stenosis from the common hepatic duct to bilateral hepatic ducts. We could not confirm bile duct malignancy, however, hilar cholangiocarcinoma was most suspicious. We performed right trisegmentectomy of the liver with caudate lobectomy and lymph node dissection 3 weeks after right portal embolization. Pathological findings confirmed localized primary sclerosing cholangitis. Surgical resection of the affected bile duct is most effective in localized primary sclerosing cholangitis, because the prognosis of the disease is poor and secondary carcinogenesis in primary sclerosing cholangitis has high incidence.