Aggressive Angiomyxoma in Males:A Report of Four Cases

Abstract

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

Aggressive Angiomyxoma

“…Aggressive angiomyxoma has been reported in the scrotum, spermatic cord, and perineum and has even been discovered incidentally during inguinal hernia surgery. 6 …”

“…[3][4][5] In men, AAM involves analogous sites, including the inguinoscrotal region and the perineum. 6 At initial clinical evaluation, AAM is often mistaken for more-common, superficial lesions, such as vaginal or labial cysts and lipomas, and the frequently large, bulky size and extent of the deep tissue involvement is not appreciated until radiographic imaging and surgical resection are performed. Histologic examination reveals a paucicellular tumor consisting of bland spindle cells in a myxoid background studded with blood vessels of varying calibers.…”

A solitary encapsulated pelvic aggressive angiomyxoma

“…[1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20] Its radiological features have been described, being a displacing mass rather than infiltrating, with high signal intensity on T2-weighted images, 1,14,15 but these features can be attributed to the myxoid component of the tumour, shown by many other soft tissue tumours of mesenchymal origin. On gross examination, it was different in being well circumscribed non-lobulated and encapsulated, [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20] but the gelatinous appearance, greyish discoloration on cross section and the large size of the tumour are common features of AAMs. These findings are attributed to the myxoid stroma that is rich in collagen fibres.…”

“…4,18 Complete excision is the treatment of choice. [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20] We believe that 1-2 yearly intervals of follow-up with alternate MRI and sonography is sufficient in view of the slow growing and nonmetastasising nature of this tumour.…”

“…[1][2][3][4][5][6][7][8] It affects women 7 times as often as men. 9,10 It was also reported in children as young as 11 years old of both sexes. 11 It has a predilection to occur in the pelvic and perineal organs.…”