A Case of Wilson's Disease Compilcated by Hepatocellular Carcinoma

Kim, Eun Joo; Kim, Chul; Chung, Il Kwon; Kim, Hong Soo; Park, Sang Hum; Lee, Moon Ho; Kim, Sun Joo; Kim, Dae Jung; Nam, Ki Ryong

Abstract

Wilson' s disease is an autosomal inherited disorder of copper metabolism; impaired copper excretion into the bile leads to copper accumulation in liver, brain and other organ. Hepatocellular carcinoma (HCC) has rarely been reported as a sequele of Wilson' s disease. It has been shown experimentally that copper has an inhibitory effect on the factors responsible for HCC development in rats, and the low prevalance of HCC in case with Wilson' s disease may result from hepatic copper deposition which has been postulated to have a protective effect against hepatic oncogenesis. The clinica features of these cases are characterized by relatively advanced age at diagnosis, the presence of cirrhosis and a male predominance. It is suggested that hepatic copper content in the patient with Wilson' s disease who was treated with penicillamine and subsequently developed HCC may be decreased toward a nonprotective range in response to penicillamine therapy. We report the one case of patient with Wilson' s disease combined with HCC.