Aggressive Angiomyxoma of the Vulva in a Teenager, a Case Report and Review of Literature

Aggressive Angiomyxoma (AA) is a rare, locally aggressive mesenchymal neoplasm that commonly involves the vulvoperineal region of females in reproductive age [1]. Steeper and Rosai first mentioned the term “aggressive angiomyxoma” in 1983 and considered it to be a rare benign mesenchymal tumor with a locally aggressive nature [9-11]. Aggressive angiomyxoma commonly presents as a painless, gelatinous soft mass of varying size [1]. It carries a high risk of local recurrence and can rarely metastasize [12]. Aggressive Angiomyxoma is often misdiagnosed because it is such a rare tumor. Therefore it should always be included in the differential diagnosis of vulvar masses. The rate of misdiagnosis varies from about 70-100% [2]. It must also be distinguished from a variety of other more common benign and malignant myxoid tumors. Along with this, it should also be differentiated from Bartholin cysts, Gartner duct cyst and from a vulval lipoma [3]. The differential diagnosis should include neurofibromas, desmoids tumors, leiomyomas, sarcomas and levator hernia [4,5].