Case Report
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World J Gastroenterol. Apr 7, 2013; 19(13): 2110-2113
Published online Apr 7, 2013. doi: 10.3748/wjg.v19.i13.2110
Hepatocellular carcinoma in a non-cirrhotic patient with Wilson's disease
Raphael Thattil, Jean-François Dufour
Raphael Thattil, Jean-François Dufour, Department of Hepatology, University Clinic of Visceral Surgery and Medicine, Inselspital University Hospital Bern, CH-3010 Bern, Switzerland
Jean-François Dufour, Division of Hepatology, Department of Clinical Research, University of Bern, CH-3010 Bern, Switzerland
Author contributions: Thattil R acquired data; Dufour JF analysed the case; Thattil R and Dufour JF wrote the paper.
Correspondence to: Dr. Jean-François Dufour, Professor, Department of Hepatology, University Clinic of Visceral Surgery and Medicine, Inselspital University Hospital Bern, CH-3010 Bern, Switzerland. jean-francois.dufour@ikp.unibe.ch
Telephone: +41-31-638026 Fax: +41-31-6329765
Received: July 22, 2012
Revised: December 3, 2012
Accepted: December 15, 2012
Published online: April 7, 2013
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.

Keywords: Wilson’s disease, Hepatocellular carcinoma, Hepatocarcinogenesis, Copper, Liver, Fibrosis, Cirrhosis