Aggressive angiomyxomas are rare, locally infiltrative mesenchymal tumors that predominantly involve the pelvic or perineal region in women. The exact etiology is unknown but is thought to be associated with a chromosomal translocation causing overexpression of the HMGA2 (high mobility group AT-hook 2) gene. This gene produces HMG proteins which are thought to act as a regulator of transcription in adipogenesis and mesenchymal differentiation.

Aggressive angiomyxomas most commonly occur in premenopausal women, although tumors in postmenopausal women and scrotal or perineal tumors in men have been documented. They typically present as an asymptomatic insidiously growing mass. Aggressive angiomyxomas are known to infiltrate local structures, but distant metastasis is rare. Reported local recurrence after surgical resection is 35%-72%.

Aggressive angiomyxomas have been associated with estrogen receptor and progesterone receptor positivity, making them susceptible to hormonal stimulation.