The challenge of pelvic aggressive angiomyxoma: case report and literature review

Abstract

Aggressive angiomyxoma is a rare, histologically benign mesenchymal tumour with infiltrative growth and a high risk of local recurrence, particularly when involving deep pelvic structures. It predominantly affects women of reproductive age and typically arises in the pelviperineal region. Tumour expression of estrogen and progesterone receptors supports a hormonally mediated pathogenesis. We report the case of a 44-year-old woman with a progressively enlarging vulvar–pelvic mass. MRI suggested aggressive angiomyxoma, later confirmed histologically. Surgical excision was performed; however, negative margins could not be achieved because of the tumour’s proximity to critical pelvic organs. Given the hormone-receptor positivity and the high likelihood of residual microscopic disease, adjuvant hormonal suppression with GnRH analogues and aromatase inhibitors was initiated. The patient remains asymptomatic and without radiologic evidence of recurrence on serial MRI during short- to medium-term follow-up. This case illustrates the diagnostic challenges of this uncommon tumour and the difficulty of achieving complete resection without compromising pelvic function. It also underscores the potential role of hormonal therapy as an adjunctive strategy, particularly in premenopausal women, although current evidence remains limited. Long-term monitoring is essential due to the tumour’s indolent course and tendency to recur.