Aggressive (deep) angiomyxoma of the vulva: a case report

Aggressive angiomyxoma of the vulva is a benign, slow-growing tumor originating from myxoid cells of connective tissue. The tumor is known for multiple local recurrences with a low tendency to metastasize. Only around 350 cases have been documented in the scientific literature so far.

We report a case of a 40-year-old North Indian, unmarried woman with a swelling on the left labium majora for 7 years, along with surface ulceration over the mass. Local examination showed a well-circumscribed, 8 × 8 cm pedunculated  mass arising from the left labium majora with an overlying ulcer measuring 6 cm × 4 cm. After taking informed written consent, wide local excision of the mass and surrounding margins was done under anesthesia. Histopathology was suggestive of aggressive angiomyxoma. Immunohistochemistry was done, which was positive for estrogen and progesterone receptors. Her postoperative recovery was uneventful. The patient was given three doses of gonadotropin-releasing hormone agonist (injection, leuprolide 3.75 mg) once a month. No recurrence has been reported so far on follow-up visits for 1 year.

Aggressive angiomyxoma is one of the differential diagnoses for vulvovaginal growth in a female. As the tumor is well known for local recurrences, correct diagnosis and appropriate management using a multidisciplinary approach are crucial to managing such patients.