Hepatocellular carcinoma in a non-cirrhotic patient with Wilson's disease

Thattil, Raphael; Dufour, Jean-François (2013). Hepatocellular carcinoma in a non-cirrhotic patient with Wilson's disease. World journal of gastroenterology, 19(13), pp. 2110-2113. WJG Press 10.3748/wjg.v19.i13.2110

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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.

Item Type:

Journal Article (Further Contribution)

Division/Institute:

04 Faculty of Medicine > Department of Gastro-intestinal, Liver and Lung Disorders (DMLL) > Clinic of Visceral Surgery and Medicine > Hepatology

UniBE Contributor:

Dufour, Jean-François

Subjects:

600 Technology > 610 Medicine & health

ISSN:

1007-9327

Publisher:

WJG Press

Language:

English

Submitter:

Lilian Karin Smith-Wirth

Date Deposited:

16 Jun 2014 15:55

Last Modified:

19 Dec 2014 19:15

Publisher DOI:

10.3748/wjg.v19.i13.2110

Uncontrolled Keywords:

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

BORIS DOI:

10.7892/boris.53739

URI:

https://boris.unibe.ch/id/eprint/53739

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