A case of rare epithelioid sarcoma developed in external genitalia.

Epithelioid sarcoma is rare neoplasm and was first recognized as distinctive entity in 1970 by Enzinger. This tumor occures cheifly in young adult and is characterized by a slowly enlarging nodule growth with early necrosis and ulceration of the overlying skin. Only 13 cases of vulvar epithelioid sarcoma have been reported. In this paper we describe the case of epithelioid sarcoma of the vulva.A 55-year-old woman was presented with a small ulcerated lesion in diameter with 2 cm painless subcutaneous nodule in left labium major, she had noticed it since 10 years before. An excisional biopsy revealed it epithelioid sarcoma. The treatment consisted of wide local excision and bilateral inguinal lymphnode dissection. There is no residual tumor, and no lymphnode metastasis. Histologically, there was neither residual tumor nor lymphnode metastasis, no further treatment was done.Although follow-up period is short, there has been no evidence of local recurrence of metastasis.