Authors: E J Heffernan M M Hayes F O Alkubaidan P W Clarkson P L Munk
Publish Date: 2008/03/13
Volume: 37, Issue: 7, Pages: 673-678
Abstract
Aggressive angiomyxoma is a rare tumour that typically occurs in the perineum in women of reproductive age A small number of cases occurring in men have been reported all of which were located in the low pelvis perineum or scrotum While benign the tumour is locally infiltrative and consequently has a high rate of local recurrence following surgery therefore accurate preoperative diagnosis is important The characteristic location of these tumours in the low pelvis or perineum has led to speculation that aggressive angiomyxomas arise from a mesenchymal cell that is unique to the perineum We describe a case of aggressive angiomyxoma arising in the thigh of a 54yearold man which we believe is the first reported instance of this rare neoplasm occurring remote from the pelvis or perineum in a male patient Crosssectional imaging demonstrated a welldefined mass that had low density on CT and high intensity on fluidsensitive MR sequences Biopsy was nondiagnostic and excision was performed At histological analysis the tumour exhibited the characteristic features of aggressive angiomyxoma with bland spindle cells and large hyalinised blood vessels in a hypocellular myxoid matrix Extensive immunohistochemical staining further supported the diagnosis While the imaging features of these tumours are nonspecific and suggestive of myxoid neoplasms the diagnosis should be considered whenever biopsy of a myxoidappearing mass yields hypocellular nondiagnostic material despite adequate sampling
Keywords: