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Turkish Journal of Cancer
2006, Volume 36, Number 1, Page(s) 031-034
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Malignant peripheral nerve sheath tumor of the vulva: A case report
ESİN ÖZEL1, HADİCE ELİF PEŞTERELİ1, TAYYUP ŞİMŞEK2, FATMA ŞEYDA KARAVELİ1, BİLAL TRAK2
1Akdeniz Unıversıty, Pathology, Antalya, Turkey
2Akdeniz Unıversıty, Obstetrıcs And Gynaecology, Antalya, Turkey

Malignant peripheral nerve sheath tumors (MPNST) constitute about 10% of soft tissue sarcomas and most MPNSTs arise in association with major nerve trunks, including the sciatic nerve, brachial plexus, and sacral plexus. The most common anatomical sites include the upper and lower extremities and trunk and less commonly the head and neck. Only few cases have been reported in the female genital tract. Our case is 63 years old and reported as epitheloid type MPNST in the left labium majus. The epitheloid type MPNST is seen extremely rare and displays some common features with carcinoma and melanoma. Cords of epitheloid cells grouped in a nodular fashion were seen in the hematoxylin-eosin stained sections of the well circumscribed, lobulated tumor. Immunohistochemically, the tumor showed positive reaction with S-100 protein, vimentin, EMA and high molecular weight keratin. There was also positive immune reaction with desmin and HMB-45 in the dedifferentiated areas of the tumor. As the tumor had no relation with the surface epithelium, it was distinguished from malignant melanoma. The case was reported as epitheloid type malignant peripheral nerve sheath tumor. [Turk J Cancer 2006;36(1):31-34].

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