Aggressive Angiomyxoma of the Vulva: A Diagnostic Dilemma

Aggressive angiomyxoma is a rare mesenchymal tumor involving soft tissue of the vulvovaginal and perineal region in females of reproductive age. A 42-year-old, para-2 female presented with an elongated swelling on the left labia majora for 10 years. She presented to us because she was feeling uncomfortable while sitting. Local examination showed an elongated mass measuring 8 x 6 cm in the longitudinal direction protruding from the lower part of the left labia majora. The mass was soft, spongy, and non-tender without any involvement of adjacent structures. Sonography of the local area showed an echogenic soft-tissue mass with increased color flow, likely a lipoma of the vulva. She underwent a wide local excision of the tumor. On histopathology, it was diagnosed as an aggressive angiomyxoma of the vulva. Angiomyxoma arises from the mesenchymal tissue, and it is locally invasive with a high recurrence rate. It has to be differentiated from Bartholin cyst, lipoma, Gartner cyst, and pelvic hernia. The diagnosis can be aided by imaging modalities such as ultrasonography, magnetic resonance imaging, and computed tomography. Wide excision is the treatment of choice. Histology, along with immunohistochemistry, can confirm the lesion. Long-term follow-up is needed as recurrences are high.