Abstract

Background: Aggressive angiomyxoma is a rare soft tissue mesenchymal tumor, locally infiltrative with a tendency to repeated local recurrence. The first description was in 1983. Normally it occurs in the vulvovaginal region, perineum and pelvis of females in reproductive age. Local wide excision of the tumor is the primary management. The local recurrence rate is high. The expression of estrogen and progesterone receptors in aggressive angiomyxoma suggests a hormone dependency of the tumor

Case report: A 50-year-old woman reported first in 2013 with a painless swelling at the perineum. She underwent surgical excision. Pathologic findings reported the tumor as an aggressive angiomyxoma. The patient did not appear to the follow-up examinations. In 2016 she reported again with a painless swelling at the perineum and underwent a local wide resection. The perineal body had to be reconstructed because of the deep infiltration of the tumor.

Conclusion: Aggressive angiomyxoma is a rare disease, but when treating women with a painless swelling in the vulvovaginal region, perineum or pelvis aggressive angiomyxoma should be considered as a differential diagnosis. There is no standardised therapy described, but complete resection seems to be important. Descriptions of pharmacologic treatment exist with gonadotropinreleasing hormone agonists.