Aggressive angiomyxoma of the perineum and pelvis

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.…”

“…[1][2][3][4] The diagnosis is based on histopathological and immunohistochemical studies. In this case, the finger-like projections into surrounding tissues was not seen, [1][2][3][4][5][6][7][8] but the low cellularity in the myxoid stroma with numerous vessels of varying wall thickness along with the spindle-shaped and stellate-shaped cells with regular nuclei and absence of mitotic figures, added to the Immunohistochemical features, are all typical for AAM. [1][2][3][4][5][6][7][8] The documented studies for immunohistochemical data in two series showed nearly 97% positive response to desmin, 94% for smooth muscle actin, 93 and 90% for oestrogen and progesterone receptors, respectively, but 0% for S100 protein.…”

“…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.…”

“…Four cases only have been reported from the UK. 6,7 We have found reports of involvement of every pelvic organ, including the urinary bladder 12 and uterus. 13 The true origin of this tumour is unknown, but few articles have raised the possibility of an isolated mesenchymal cell origin.…”