Angiomyofibroblastoma of the vulva is a rare mesenchymal tumor. It has been diagnostically confused with aggressive angiomyxoma which has a somewhat different clinical course and therapy. Herein we report a case of angiomyofibroblastoma of the vulva in a 46-year-old female.
Microscopically, there were alternating hypercellular and hypocelluar edematous zones in which abundant capillary blood vessels were irregularly distributed. Spindle, plump spindle, and oval stromal cells were concentrated around the blood vessels, or loosely dispersed in the hypocellular area. Immunohistochemically, the stromal cells were positive for desmin, vimentin, muscle-specific actin and weakly positive for S-100 protein. Ultrastructural studies showed well developed rough endoplasmic reticulum, abundant intermediate filaments, and pinocytic vesicles in the stromal cells.
Angiomyofibroblastoma is a distinctive, hitherto uncharacterized, benign soft tissue tumor of the vulva with histology similar to an aggressive pelvic angiomyxoma. It can be distinguished from an aggressive angiomyxoma by its circumscribed borders, higher cellularity, more numerous blood vessels, the frequent presence of plump stromal cells, minimal stromal mucin, and rarity of erythrocyte extravasation. We experienced a case of angiomyofibroblastoma of the vulva occurring in a 45-year-old woman. The lesion was a well-defined but not encapsulated mass, 4.5x4.2 cm. Histologically the mass was characterized by alternating hypercellular and hypocellular edematous zones in which abundant blood vessels were irregularly distributed. Immunohistochemically, the spindled, plump spindled, and oval stromal cells were reactive for vimentin and desmin, but not for cytokeratin, or S-100 protein.
Angiomyofibroblastoma is a rare, benign mesenchymal tumor of the vulva. Since it was described in 1992 by Fletcher, 15 cases have been reported in literature. We recently experienced a recurred angiomyofibroblastoma of the vulva.
A 45-year-old woman was presented initially in 1991 with a mass of labium major and local excision of tumor mass had been performed. A histologic diagnosis was made of angiomyxoma, but this diagnosis was revised to angiomyofibroblastoma by the authors. The recurred mass was well circumscribed, measuring 2.5x1.6x1.5cm in dimensions.
Microscopically the tumor was characterized by high cellularity, numerous blood vessels(which lack prominent hyalinization), and plump stromal cells.
Immunohistochemically, the stromal cells were reactive for vimentin and desmin, but not alpha-smooth muscle actin, or S-100 protein. We thought that this case was a recurred angiomyofibrblastoma of the vulva due to incomplete surgical excision.