Aggressive angiomyxoma of the vulva

Aggressive angiomyxoma is a rare soft tissue tumour that carries a high risk of local relapse. It is a slowly growing and locally infiltrating tumour. We describe the case of an aggressive pelvic-perineal angiomyxoma arising in a 36-year-old woman. The patient had a mass that grew before, during and after her pregnancy. Transperineal surgery was performed. The resection margins were free of disease. Our case confirms what has previously been published concerning the possible hormone-dependence of this neoplasm. Given the positive estrogen and progesterone receptor status of this tumour, we will consider hormonal treatments in the case of a future relapse.