Late Recurrence of Aggressive Angiomyxoma of the Vulva

Abstract

Aggressive angiomyxoma (AA) is found mainly in the pelvis and perineum, with a female-to-male ratio of approximately 6:1. Although it is a slow growing tumor, AA has a marked tendency to local recurrence with a low metastasis capacity. The study aimed to describe a case of vulvar angiomyxoma recurred almost 20 years after its initial surgery. We report the case of a 57-year-old gravida 5 para 4 woman with vulvar AA arising from the left labium majus, which recurred 20 years after initial surgery. There was a nontender, solid, mobile mass on the left vulva, which was 25 x 30 cm on physical examination. A pelvic computed tomographic scan showed a mass measuring 26 x 10 x 14 cm originating from left vulvar region, which has a fatty tissue density. Under general anesthesia, total excision of the tumor was performed. Macroscopically, the tumor weighed 723 g and measured 33 x 20 x 10 cm. The histopathological examination of the specimen revealed a myxoid tumor with sparse infiltrates of spindle-shaped to stellate cells and vessels of varying sizes. The final histopathological diagnosis was AA. Aggressive angiomyxoma may form extremely large tumors, and recurrence is not rare even many years after primary surgery.