Abstract

The smooth muscle tumors are rare lesions of the vulva that represents a group including the tumors of uncertain malignant potantial.In this paper a case of epithelioid smooth muscle tumor of uncertain malignant potantial of the vulva in a 16 year old girl was encountered.The lesion was a well circumscribed solitary mass measuring up to 14cm Microscopically the lesion was composed of moderately pleomorphic epithelioid cells. The mitotic count is variable,the most mitotic active area showed 4-5 mitosis in 10 high power fields with an avarage of 1 per 10 high power fields.Immunohistochemically the tumor is immunoreactive for smooth muscle markers.Due to the great tumor dimension,moderately pleomorphic epithelioid cells and immunohistochemical profile of the tumor, the final histopatologic diagnosis of epithelioid smooth muscle tumor of uncertain malignant potantial was made.The lump was excised with negative surgical margins. The follow up information was available for 18 months and there was no recurrence or metastasis. This case gives us the opportunity to review the clinical and pathologic features of this rare entitiy determining the pathologic malignant criteria.