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Verfasst von:Pfeiffenberger, Jan [VerfasserIn]   i
 Mogler, Carolin [VerfasserIn]   i
 Gotthardt, Daniel [VerfasserIn]   i
 Schulze-Bergkamen, Henning [VerfasserIn]   i
 Schemmer, Peter [VerfasserIn]   i
 Schirmacher, Peter [VerfasserIn]   i
 Stremmel, Wolfgang [VerfasserIn]   i
 Weiss, Karl Heinz [VerfasserIn]   i
Titel:Hepatobiliary malignancies in Wilson disease
Verf.angabe:Jan Pfeiffenberger, Carolin Mogler, Daniel N. Gotthardt, Henning Schulze-Bergkamen, Thomasz Litwin, Ulrike Reuner, Harald Hefter, Dominik Huster, Peter Schemmer, Anna Członkowska, Peter Schirmacher, Wolfgang Stremmel, David Cassiman, and Karl Heinz Weiss
Jahr:2015
Umfang:8 S.
Fussnoten:First published: 23 November 2014 ; Gesehen am 05.07.2017
Titel Quelle:Enthalten in: Liver international
Ort Quelle:Oxford : Wiley-Blackwell, 2003
Jahr Quelle:2015
Band/Heft Quelle:35(2015), 5, Seite 1615-1622
ISSN Quelle:1478-3231
Abstract:Backgrounds & Aims 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. Methods 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. Results Of a total of 1186 patients, fourteen developed hepatobiliary malignancies. Eight were hepatocellular carcinomas (HCC) and six were intrahepatic cholangiocellular carcinomas (ICC). 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. Conclusions The rate of hepatobiliary malignancies in Wilson disease is very low, even in cirrhotic patients. As a result of the relevant number of ICC in addition to HCC 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-specific medical treatment on tumour activity remains to be elucidated.
DOI:doi:10.1111/liv.12727
URL:Bitte beachten Sie: Dies ist ein Bibliographieeintrag. Ein Volltextzugriff für Mitglieder der Universität besteht hier nur, falls für die entsprechende Zeitschrift/den entsprechenden Sammelband ein Abonnement besteht oder es sich um einen OpenAccess-Titel handelt.

Volltext ; Verlag: http://dx.doi.org/10.1111/liv.12727
 Volltext: http://onlinelibrary.wiley.com/doi/10.1111/liv.12727/abstract
 DOI: https://doi.org/10.1111/liv.12727
Datenträger:Online-Ressource
Sprache:eng
Sach-SW:ATP7B
 hepatobiliary malignancies
 liver cancer
 Wilson's disease
K10plus-PPN:1560474955
Verknüpfungen:→ Zeitschrift

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