Small-duct primary sclerosing cholangitis with hepatocellular carcinoma requiring liver transplantation

BACKGROUND:Primary sclerosing cholangitis(PSC)is a chronic progressive cholestatic liver disease,which usually affects young adults and is diagnosed by cholangiography.On a few occasions,the disease either starts in or exclusively involves the small intrahepatic bile ducts,referred to as small-duct PSC. METHODS:A 31-year-old man presented with severe hematemesis secondary to liver cirrhosis.Over a course of 8 years,his liver decompensated and required an orthotopic liver transplantation. In this report we discuss his disease presentation,course of management,and the post-transplantation course of manage- ment,and review the morphologic diagnosis,and differential diagnosis of the disease with large-duct type and other diseases that involve small intrahepatic bile ducts. RESULTS:The patient’s explanted liver showed changes of PSC affecting only the small-and medium-sized bile ducts in addition to three incidental nodules of hepatocellular carcinoma. CONCLUSIONS:Small-duct PSC has a substantially better prognosis than the large-duct type,with less chance of developing cirrhosis and an equal risk for developing hepato- cellular carcinoma,but no increased risk for developing cholangiocarcinoma.Treatment seems to help relieve the symptoms but not necessarily improve survival.Liver transplantation remains the ultimate cure.