Zusammenfassung
Das epitheloide Sarkom der Vulva ist ein seltener aggressiver maligner Weichgewebstumor. In der Regel sind junge Patientinnen betroffen. Der Tumor zeigt typische histomorphologische und immunhistologische Charakteristika. Aufgrund seiner bevorzugten Lokalisation in der Region der Bartholin'schen Drüsen kann das epitheloide Vulvasarkom mit einem Entzündungsprozess in diesem Bereich verwechselt werden. Die histologische Diagnose kommt dann überraschend. Aufgrund der hohen Rezidiv- und Metastasierungsneigung ist es entscheidend, den Tumor früh zu erkennen und zu therapieren. Dabei ist die chirurgische Tumorexzision im Gesunden die wichtigste therapeutische Maßnahme.
Abstract
Primary epitheloid sarcomas of the vulva are rare but aggressive soft-tissue tumors. Most occur in young women and are located in the region of Bartholin's gland. The histomorphologic and immunohistochemical features of the tumor are characteristic. The differential diagnosis includes inflammatory conditions of Bartholin's gland. The tumors tend to recur locally. Wide local excision is the treatment of choice.
Schlüsselwörter
Epitheloides Sarkom - Vulva
Key words
Epitheloid sarcoma - Vulva - Vulvar sarcomas
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Angela Wohlfarth
Institut für Pathologie Klinikum Nürnberg
Prof.-Ernst-Nathan-Str. 1
90340 Nürnberg
