Aggressive angiomyxoma of the female pelvis and perineum: a case series

Abstract

Aggressive angiomyxoma (AA) was first described in 1983, and fewer than 150 cases have been reported in the world medical literature. These tumors are benign, locally infiltrative mesenchymal neoplasms with a predilection for the female pelvis and perineum and a tendency to recur. The size of AAs at presentation varies considerably; however, these tumors often achieve large dimensions before becoming clinically symptomatic. Surgical excision remains the mainstay of treatment, but whether clear, tumor-free surgical margins are necessary is controversial. We report a cohort of six patients treated surgically during the past 20 years for primary or recurrent AA. Treatment, surgical margin status, estrogen and progesterone receptor status, and outcomes are reviewed.