[Aggressive angiomyxoma of the vulva presenting as a huge pedunculated tumor].

In 1983, Steeper and Rosai described aggressive angiomyxoma as a rare, slow growing, locally infiltrative, soft tissue tumor that occurs mainly in the genital, perineal and pelvic region, mostly in women. Usually this tumor is nonmetastasing. Recurrence is frequent in about 30%-72% cases. Angiomyxoma demonstrates estrogen and progesterone receptor positivity. Primary surgical resection is the treatment of choice. Hormonal therapy with tamoxifen, raloxifene, and gonadotropin-releasing hormone analogs (GnRH agonists) and vascular embolization are used as the treatment of recurrence and may help to make complete excision feasible in large tumors. We report a case of a 43-year-old woman with huge pedunculated aggressive angiomyxoma of the vulva treated with primary excision.