Aggressive angiomyxoma of the vulva

Abstract

Introduction: Aggressive angiomyxoma (AAM) is a rare vulvovaginal, perineal or pelvic mesenchymal neoplasm that occurs almost exclusively in women of reproductive age.

Clinical case: We report a case of an aggressive angiomyxoma of vulva in a 33-years-old women, with progressive growth for six years, with rapid growth during pregnancy. On physical examination, a tumor of 7x5cm was found, located on the right labia majora, soft tissue dependent. Ultrasound-guided needle biopsy was performed, but the diagnosis couldn’t be made. Wide resection of the tumor was performed at 19 weeks pregnant. Hystopathology reported an aggressive angiomyxoma of the vulva, with positive estrogen and progesterone receptors and with tumor in resection margins. Due to being pregnant, the decision of surveillance was taken. Adjuvant therapy was not performed and there is no recurrence until now.

Discussion: At present, surgery remains the main treatment option for AAM. A retrospective study with the largest number of cases (106 cases) pointed out that there was no significant difference in the recurrence-free rate between patients with negative and positive margins within 10 years after surgery. Adjuvant radiotherapy and hormonal therapy are not fully stablished.

Conclusion: Aggressive angiomyxoma is a rare neoplasm. The treatment is surgical resection. As late recurrences are known, all patients need long-term follow-up. Periodic clinical examination may be insufficient to detect recurrence. Imaging studies, such as MRI, may detect recurrence early, but there are no guidelines about their frequency.