title: No increased risk of hepatocellular carcinoma in cirrhosis due to Wilson disease during long-term follow-up.
abstract: OBJECTIVE
  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.
authors.affiliation: Department of Gastroenterology and Hepatology, University Medical Center, Utrecht, The Netherlands.
authors.forename: Suzanne
authors.initials: S
authors.lastname: van Meer
authors.forename: Robert A
authors.initials: RA
authors.lastname: de Man
authors.forename: Aad P
authors.initials: AP
authors.lastname: van den Berg
authors.forename: Roderick H J
authors.initials: RH
authors.lastname: Houwen
authors.forename: Francisca H H
authors.initials: FH
authors.lastname: Linn
authors.forename: Martijn G H
authors.initials: MG
authors.lastname: van Oijen
authors.forename: Peter D
authors.initials: PD
authors.lastname: Siersema
authors.forename: Karel J
authors.initials: KJ
authors.lastname: van Erpecum
mesh_terms: D000293:Adolescent; D000328:Adult; D006528:Carcinoma, Hepatocellular / Q000453:epidemiology / Q000209:etiology*; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D005500:Follow-Up Studies; D006527:Hepatolenticular Degeneration / Q000150:complications*; D006801:Humans; D007223:Infant; D008103:Liver Cirrhosis / Q000209:etiology*; D008113:Liver Neoplasms / Q000453:epidemiology / Q000209:etiology*; D008297:Male; D012189:Retrospective Studies; D012306:Risk; D018570:Risk Assessment; D012720:Severity of Illness Index; D013997:Time Factors; D055815:Young Adult
keywords: Wilson disease; cirrhosis; hepatocellular carcinoma