Giant Aggressive Angiomyxoma of Vulva: A Rare Entity with Literature Review

Aggressive Angiomyxoma (AA) is a rare locally aggressive mesenchymal tumor, which is usually seen occurring in the females in reproductive age group involving predominantly pelvic, perineal and vulvovaginal regions. We describe a case of a 40 year old female presenting with painful swelling in perianal region. The tumor was excised completely with free margins and final diagnosis was made on histopathological examination. On immunohistochemical examination, tumor cells are positive for oestrogen and progesterone receptor. Early diagnosis, surgical resection and long term follow up is required as its recurrence rate is high but very low chance of distant metastasis.