Angiomyofibroblastoma of the vulva: a case report of a pedunculated variant and review of the literature.

OBJECTIVE: Angiomyofibroblastoma (AMF) is a benign mesenchymal tumor usually found in the vulva. We reviewed 70 cases of vulvar AMF that have been reported in the English-language literature and report 1 case of a pedunculated variant. Our case brings the total reported to 71 and is only the fourth pedunculated variant reported. METHODS: This 50-year-old woman presented to our gynecology clinic with a 1-year history of a left labial mass. It began as pea-sized, and rapidly grew to 12 cm in diameter. Physical examination demonstrated a 12-cm pedunculated soft mass arising from the left labia majora. The clinical diagnosis was aggressive angiomyxoma, and a simple excision was performed. The final pathology demonstrated AMF. The patient remains free from tumor at 4 years of follow-up. RESULTS: Seventy-one cases were summarized. The mean age at presentation was 45 years. The lesions were equally distributed between the left (52%) and right (48%). The most common clinical diagnosis was a Bartholin gland cyst (46%) or lipoma (15%). The mean duration of the lesion before seeking treatment was 29 months, and the mean diameter at presentation was 5.9 cm. All of the patients were treated with simple excision. The mean duration of follow-up was 37 months. There was 1 report of sarcomatous transformation 2 years after initial treatment. CONCLUSIONS: Angiomyofibroblastoma is a rare benign tumor that most often occurs in the vulva. Differential diagnosis may include aggressive angiomyxoma, Bartholin cyst, or lipoma. The treatment of choice is simple total excision, which is usually curative.