Aggressive angiomyxoma of the thigh

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 pre-operative 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 54-year-old man, which we believe is the first reported instance of this rare neoplasm occurring remote from the pelvis or perineum in a male patient. Cross-sectional imaging demonstrated a well-defined mass that had low density on CT and high intensity on fluid-sensitive MR sequences. Biopsy was non-diagnostic 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 non-specific and suggestive of myxoid neoplasms, the diagnosis should be considered whenever biopsy of a myxoid-appearing mass yields hypocellular, non-diagnostic material, despite adequate sampling.

Angiomyxoma of thigh in a young lady: rare presentation

“…Angiomyxoma patients are usually asymptomatic due to the slow and insidious growth pattern of the tumor. 5 As previously reported the female to male ratio is approximately 6.6:1. Steeper and Rosai reported the locally aggressive behavior of the tumor and its tendency to recur locally.…”

Aggressive Angiomyxoma: an Unusual Presentation

“…This is likely due to the tumor's high water content and loose myxoid matrix (9). The tumor shows moderate enhancement following intravenous contrast administration, which is related to its inherent vascularity (9). The recurrent tumor showed in the present case had a similar appearance to the primary lesion reported before.…”

Aggressive angiomyxoma of the thigh: A case report and review of the literature

“…In the present study, the mass was hypodense with no invasion of the skeletal structures and no hydrops in the articular cavity. Enhanced CT demonstrated high vascularity in the AAM mass (5). The characteristic imaging of AAM on MRI is isointense relative to muscle on T1-weighted images and hyperintense on T2-weighted images (14).…”

“…AAM has also been described in the male larynx, genital tract and supraclavicular fossa (2)(3)(4). A notable case of AAM on the upper, lateral aspect of the right thigh in a male patient has also been reported (5). Due to the high recurrence rate, tumor excision with wide tumor-free margins should be performed for AAM.…”