Abstract

Background: Aggressive angiomyxoma (AA) is a rare, locally aggressive mesenchymal neoplasm that commonly involves the vulvoperineal region of females in reproductive age [1]. AA commonly presents as a painless, gelatinous soft mass of varying size [1]. It carries a high risk of local recurrence and can rarely metastasize [1]. 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]. The treatment of choice for aggressive angiomyxoma is surgical excision with wide margins to help prevent local recurrences [6]. A perineal surgical approach is often most successful. The tumor can recur at any time, therefore close monitoring of the patient is necessary following surgery. Case: We present a case of aggressive angiomyxoma of the vulva in a 20-year-oldcelibate female. The patient underwent wide local excision of the tumor with clear margins. Conclusion: Aggressive angiomyxoma (AA) is a mesenchymal tumor that most commonly occurs in women between the ages of 31-35 years old [3]. The tumor usually occurs in either the perineal or pelvic region and typically presents as a slow growing, painless mass [7]. AA is considered to be an aggressive tumor because it has a high risk of infiltration and local recurrence. However, it rarely metastasizes so it has an overall good prognosis [8]. This case highlights the need to consider angiomyxoma in the differential diagnosis for tumors of unknown cause in the vulvar region [7]. Even though it is a rare diagnosis, it must be identified in order to properly treat it and prevent invasion of local structures. Histopathology is the key to correctly diagnosing this mass. Magnetic resonance imaging (MRI) can help provide additional information about the size and extent of the tumor as well. Surgical excision of the tumor with wide margins to prevent local recurrence is the treatment of choice [6]. Alternative treatments such as GnRH agonists can aid in treatment as well by decreasing the size of the tumor prior to surgery. Since the local recurrence rate is about 30-72%, close monitoring after surgery is extremely important.

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