Aggressive Angiomyxoma of the Vulva in Pregnancy: A Case Report and Review of Management Options

Rashmi Bagga, MD; Anish Keepanasseril, MBBS; Vanita Suri, MD; Raje Nijhawan, MD

In This Article


Aggressive angiomyxoma is a rare, locally infiltrative mesenchymal tumor found usually in women of reproductive age, and was first described by Steeper and Rosai[1] in 1983. It is a slow-growing, low-grade neoplasm involving the pelvis and perineum with a high risk for local recurrence, which occurs after many years. Therefore, initial surgical resection with wide margins followed by long-term surveillance is advised. Many options for the treatment of recurrences have been tried with varying success, but no single modality is clearly beneficial over others. Extensive and repetitive surgeries are associated with increased morbidity. Current research does not support the earlier quoted dictum of initial excision with "wide surgical margins," as the recurrence rate in patients with narrow surgical margins is not higher than that of patients with wide surgical margins.[2] Estrogen and progesterone receptors are commonly found in aggressive angiomyxoma, and so they may be hormone-dependent.[2,3,4,5,6] Hence, they are likely to grow during pregnancy and respond to hormonal manipulation. However, only a few cases of its detection and management during pregnancy are reported in the literature.[2,4,5,6] A primigravida with aggressive angiomyxoma of the vulva excised at 16 weeks of gestation is reported with the aim to discuss management options and subsequent follow-up.


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