Aggressive angiomyxoma (AAM) is a rare, locally infiltrative but nonme
tastasizing tumor of the pelvic and perineal soft tissues that occurs
almost exclusively in adult females. The authors describe four cases o
f AAM in adult males that arose in the scrotum. There was some histolo
gic variation among the tumors. One case was focally hypercellular aro
und the blood vessels, two were more densely cellular throughout, and
one had cystic degeneration. Three of the tumors were widely infiltrat
ive, and one of the four tumors recurred locally. The clinicopathologi
c features of these cases are similar to those of AAM occurring in fem
ales. In either sex, AAM should be distinguished from benign myxoid tu
mors with a low risk of local recurrence and fully malignant myxoid tu
mors with distant metastatic potential.