Zusammenfassung
Die multiple endokrine Neoplasie Typ 1 (MEN-1-Syndrom oder Wermer-Syndrom), eine seltene autosomal dominante Erkrankung, ist durch das Auftreten von pathologischen Veränderungen innerhalb mehrerer endokriner Organsysteme charakterisiert. In über 90% geht sie mit einer Hyperplasie der Nebenschilddrüsen einher, wesentlich seltener sind Hypophysenvorderlappenadenome (45–60%), endokrine Pankreastumoren (30–80%), Nebennierenrindentumoren (15–34%) und andere neuroendokrine Tumoren (5–26%). Die Klinik des MEN-1-Syndroms richtet sich nach dem betroffenen endokrinen Organsystem. Ein genetisches Screening ermöglicht die Früherkennung betroffener Familienmitglieder von MEN-1-Patienten, sowie auch die Diagnosesicherung der Erbkrankheit bei Tumoren, die MEN-1-assoziiert auftreten können. Bei Genträgern wird ein jährliches biochemisches Screening empfohlen, um die frühzeitige Diagnose weiterer benigner oder maligner MEN-1-assoziierter endokriner Tumoren zu ermöglichen.
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Dotzenrath, C. et al. (2000). Multiple endokrine Neoplasien (MEN). In: Siewert, J.R., Harder, F., Rothmund, M. (eds) Praxis der Viszeralchirurgie. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-09422-8_6
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