{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,27]],"date-time":"2026-09-27T15:21:22Z","timestamp":1790522482977,"version":"4.1.0"},"reference-count":51,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2014,11,23]],"date-time":"2014-11-23T00:00:00Z","timestamp":1416700800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Liver International"],"published-print":{"date-parts":[[2015,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Backgrounds &amp; Aims<\/jats:title><jats:p>Reports of hepatobiliary malignancies in Wilson disease are sparse. The aim of this study was to evaluate hepatobiliary malignancies in Wilson disease patients concerning the clinical course of tumour disease and pathological analysis of tumour tissue.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Multicenter cohort study of patients with confirmed diagnosis of Wilson disease treated at the Second Department of Neurology, Institute of Psychiatry and Neurology, Warsaw, Poland, the university hospitals Heidelberg, Duesseldorf and Dresden, Germany, and the Department of Hepatology, University Leuven, Belgium. Occurrence, treatment and outcome of hepatobiliary tumours were analysed retrospectively.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Of a total of 1186 patients, fourteen developed hepatobiliary malignancies. Eight were hepatocellular carcinomas (<jats:styled-content style=\"fixed-case\">HCC<\/jats:styled-content>) and six were intrahepatic cholangiocellular carcinomas (<jats:styled-content style=\"fixed-case\">ICC<\/jats:styled-content>). The prevalence of hepatobiliary malignancies in the cohort was 1.2% and the incidence was 0.28 per 1000 person years. Pathological analysis of tumour material showed no abnormal copper concentration.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>The rate of hepatobiliary malignancies in Wilson disease is very low, even in cirrhotic patients. As a result of the relevant number of <jats:styled-content style=\"fixed-case\">ICC<\/jats:styled-content> in addition to <jats:styled-content style=\"fixed-case\">HCC<\/jats:styled-content> histological analysis through surgical resection or biopsy should be mandatory when a suspect liver lesion is detected. The influence of copper depletion from Wilson disease\u2010specific medical treatment on tumour activity remains to be elucidated.<\/jats:p><\/jats:sec>","DOI":"10.1111\/liv.12727","type":"journal-article","created":{"date-parts":[[2014,11,4]],"date-time":"2014-11-04T14:49:10Z","timestamp":1415112550000},"page":"1615-1622","source":"Crossref","is-referenced-by-count":91,"title":["Hepatobiliary malignancies in Wilson disease"],"prefix":"10.1111","volume":"35","author":[{"given":"Jan","family":"Pfeiffenberger","sequence":"first","affiliation":[{"name":"Department of Internal Medicine IV University Hospital Heidelberg Heidelberg Germany"},{"name":"Liver Cancer Center Heidelberg University Hospital Heidelberg Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carolin","family":"Mogler","sequence":"additional","affiliation":[{"name":"Liver Cancer Center Heidelberg University Hospital Heidelberg Heidelberg Germany"},{"name":"Institute of Pathology University 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