title: Hepatocellular carcinoma in a non-cirrhotic patient with Wilson's disease.
abstract: We report the exceptional case of hepatocellular carcinoma in a non-cirrhotic patient, whose Wilson's disease was diagnosed at the unusual age of 58 years. The liver histology revealed macrovesicular steatosis with fibrosis, but no cirrhosis. The disease was treated with D-penicillamine for 3 years until acute discomfort in the right upper quadrant led to detection of multifocal hepatocellular carcinoma, which was successfully resected. The histological examination confirmed the malignant nature of the 4 lesions, which were classified according to Edmondson and Steiner as poorly differentiated hepatocellular carcinoma grade 3. The non-tumoral parenchyma showed 80% steatosis with ballooned cells, lobular inflammation, septal fibrosis but no cirrhosis. Hepatocellular carcinoma is rare in Wilson's disease, especially in the absence of cirrhosis. The literature's 28 published cases are reviewed and the contributory role of copper in the hepatocarcinogenic process is discussed.
authors.affiliation: Department of Hepatology, University Clinic of Visceral Surgery and Medicine, Inselspital University Hospital Bern, CH-3010 Bern, Switzerland.
authors.forename: Raphael
authors.initials: R
authors.lastname: Thattil
authors.forename: Jean-François
authors.initials: JF
authors.lastname: Dufour
mesh_terms: D001706:Biopsy; D006528:Carcinoma, Hepatocellular / Q000150:complications / Q000175:diagnosis*; D003300:Copper / Q000378:metabolism; D005091:Exons; D005355:Fibrosis / Q000175:diagnosis; D006527:Hepatolenticular Degeneration / Q000150:complications / Q000175:diagnosis*; D006801:Humans; D007249:Inflammation; D008113:Liver Neoplasms / Q000150:complications / Q000175:diagnosis*; D008297:Male; D008875:Middle Aged; D020125:Mutation, Missense; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
chemical_list: D003300:Copper
keywords: Cirrhosis; Copper; Fibrosis; Hepatocarcinogenesis; Hepatocellular carcinoma; Liver; Wilson’s disease