A persistent mass: A case of aggressive Angiomyxoma of the vulva

Abstract

We present a case of aggressive angiomyxoma of the vulva. The patient presented with a persistent, enlarging vulvar mass, initially misdiagnosed as a Bartholin gland cyst. The patient underwent wide local excision, which resulted in total resection of the mass. Final pathology was consistent with aggressive angiomyxoma, a rare soft tissue tumor with a predilection for the female pelvis. Though rare, it is important to consider in the differential diagnosis of a pelvic mass, given the locally aggressive nature of this tumor and propensity for recurrence.

Rare case of huge vulvar angiomyofibroblastoma in a young female

“…AA was introduced first by Steeper and Rosai (1983) . It was described as an infiltrating tumour with an average size of >5 cm, typically occurring at an even younger age than AMFB and with faster growth in weeks to months rather than years ( Wang et al, 2018 , Nagai et al, 2010 , Steeper and Rosai, 1983 , Brzezinska et al, 2018 ).…”

Aggressive angiomyxoma persistently misdiagnosed as an obturator hernia managed with resection and hormonal therapy: case report

“…The most encountered presentation of AA is a vaginal or vulvar soft mass, usually misdiagnosed as a Bartholin’s duct cyst, vulvar mass, lipoma, vulvar abscess, vaginal cyst, Gardner’s duct cyst, vaginal prolapse or levator hernia [ 6 ]. Misdiagnosed cases of AA have been reported to be as high as 82% [ 3 ]. Definitive diagnosis is made on the basis of histopathological features, typically stellate and spindle cells with poorly demarcated cytoplasm, separated by loose myxoid stroma abundant in collagen fibrils [ 4 ].…”

“…Aggressive angiomyxoma (AA) was first described by Steeper and Rosai in 1983 as a soft-tissue mass that mostly occurs in the pelvis and perineum, with a female-to-male ratio of ~6:1. [ 1 , 2 ] Prevalence is undetermined due to the rarity of the condition, which makes management and counseling difficult [ 3 ]. AAs are characterized by their large size, and their noncapsulated, locally infiltrative nature.…”

Aggressives Angiomyxom der vorderen Scheidenwand

“…Die chirurgische R0-Resektion ist therapeutischer Goldstandard, was jedoch bei ausgeprägtem Befund mit hoher Morbidität verbunden ist. Die Lokalrezidivrate beträgt zwischen 25 und 47 %, davon 85 % in den ersten 5 Jahren [5]. Die Metastasierung ist äußerst selten und die Prognose im Allgemeinen gut.…”