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J Gastroenterol Hepatol. 2015 Mar;30(3):535-9. doi: 10.1111/jgh.12716.

No increased risk of hepatocellular carcinoma in cirrhosis due to Wilson disease during long-term follow-up.

Author information

1
Department of Gastroenterology and Hepatology, University Medical Center, Utrecht, The Netherlands.

Abstract

BACKGROUND AND AIMS:

Data on risk of hepatocellular carcinoma (HCC) in patients with Wilson disease are scarce. We determine HCC risk in a well-defined cohort of Wilson patients.

METHODS:

All patients with a confirmed diagnosis of Wilson disease (Leipzig score ≥ 4) in three Dutch university referral hospitals were included in this retrospective cohort study. End of follow-up was defined as date of diagnosis of HCC, liver transplantation, death, or last available hospital visit. Also, a meta-analysis was performed to determine incidence and mortality rate of HCC in Wilson disease based on all published cohorts.

RESULTS:

In total, 130 patients with Wilson disease were followed during a median follow-up of 15 years (range 0.1-51.2). At baseline, cirrhosis was present in 74 patients (57% of total: 64% compensated, and 36% decompensated). At end of follow-up, liver disease severity was improved, stable or deteriorated in 20%, 46%, and 24% of all cases (10% unknown), respectively. Two patients developed HCC (one despite excellent decoppering after 50 years follow-up, the other with newly diagnosed Wilson disease). Estimated annual HCC risk for all patients was 0.09% (95% confidence interval [CI]: 0.01-0.28). Subgroup analysis in cirrhotic patients revealed an annual HCC risk of 0.14% (95% CI: 0.02-0.46). The meta-analysis showed an annual HCC risk of 0.04% (95% CI: 0.01-0.10) and HCC mortality rate of 2.6/10 000 person-years (95% CI: 0.7-7.0).

CONCLUSIONS:

Even in case of cirrhosis, HCC risk is low in Wilson disease. Our data do not support regular HCC surveillance in Wilson disease.

KEYWORDS:

Wilson disease; cirrhosis; hepatocellular carcinoma

PMID:
25160780
DOI:
10.1111/jgh.12716
[Indexed for MEDLINE]

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