RESUMO
We report a case of vulvar aggressive angiomyxoma (AA) which is a rare, slow growing and benign tumor of mesenchymal origin, but has a high risk of local recurrence. A 49-year-old Japanese female was referred to us with a large mass of the left vulva, measuring 15×9.5×9 centimeters. She underwent surgical excision of the tumor with no evidence of recurrence on a 5-year follow up. In this case, histopathological examination and immunohistochemical staining after excision revealed a diagnosis of vulvar AA with estrogen and progesterone receptors positive. Aggressive angiomyxoma of the vulva needs to be distinguished from benign myxoid tumor with a low risk of local recurrence as well as from malignant neoplasma. The first line treatment of AA is complete surgical excision with tumor free margins, it will reduce the recurrence.
