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DOI: 10.1055/s-0030-1270708
© Georg Thieme Verlag KG Stuttgart · New York
Das PECom des Uterus – ein seltener mesenchymaler Tumor: Fallbericht und Literaturübersicht
PEComa of the Uterus – A Rare Mesenchymal Tumor: Case Report and Review of the LiteraturePublication History
eingereicht 3.12.2010
revidiert 17.12.2010
akzeptiert 23.12.2010
Publication Date:
24 February 2011 (online)
![](https://www.thieme-connect.de/media/gebfra/201102/lookinside/thumbnails/10.1055-s-0030-1270708-1.jpg)
Zusammenfassung
PECome des Uterus sind seltene mesenchymale Tumoren, ausgehend von perivaskulären, glattmuskulär differenzierten Zellen mit epitheloider Morphologie und immunhistochemischer Koexpression von glattmuskulären und melanozytären Markern. Mit einer Gesamtüberlebensrate von 92 % ist die Prognose gut und die Majorität der PECome ist als „low grade“-Läsion einzuordnen. Dennoch wird ein engmaschiges Follow-up empfohlen. Die Stadieneinteilung sollte nach Art der Leiomyosarkome erfolgen. Der vorliegende Fall wurde bei einer 65-jährigen Frau beobachtet mit einer maximalen Tumorgröße von 6 cm mit Infiltration adnexaler Mesostrukturen.
Abstract
Perivascular epithelioid cell tumor (PEComa) of the uterus is a rare mesenchymal lesion characterized by a proliferation of perivascular epitheloid cells with eosinophilic cytoplasm and immunohistochemical coexpression of both smooth muscle and melanocytic markers. The majority (∼ 92 %) of PEComas exhibit a benign behavior and are considered low-grade lesions. Nevertheless, a close follow-up of patients is necessary, and tumors should be staged like leiomyosarcomas. The presented case of a 65-year-old woman revealed a PEComa with diameter of 6 cm and adnexal involvement.
Schlüsselwörter
Tumor - Uterus - mesenchymaler Tumor - PEComa
Key words
perivascular epitheloid cell tumor - PEComa - uterus - mesenchymal tumor
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Prof. Dr. med. Lars-Christian Horn
Abteilung Mamma-, Gynäko- & Perinatalpathologie
Institut für Pathologie
Universität Leipzig
Liebigstraße 26
04103 Leipzig
Email: hornl@medizin.uni-leipzig.de