Answer: Aggressive angiomyxoma

Histology: The lesion consists of a myxoid neoplasm composed of small, stellate cells with benign-appearing nuclei within a myxoid stroma. Mitotic figures are absent. Throughout the lesion are scattered vessels of different sized caliber. Small bundles of smooth muscle and thicker muscles are present within the myxoid matrix as well as around some of the vessels. The tumor is positive for estrogen receptor, progesterone receptor, and CD34 with negative stains for actin, desmin, and S-100.

Discussion: Aggressive angiomyxomas typically are found in the pelvic and perineal region in females in the third-to-sixth decade of life. They have rarely been found in men in the pelvis in sites including the epididymis, scrotum, spermatic cord, and perineum. These lesions have a tendency for slow growth, yet they are associated with a high local recurrence rate. Typically, they are cured on re-excision and these lesions have no metastatic potential.