Aggressive (deep) angiomyxoma of the vulva: a case report 

Abstract

Background Aggressive angiomyxoma of the vulva is a benign, slow-growing tumor originating from myxoid cells of connective tissue. The tumor is known for multiple local recurrences with a low tendency to metastasize. Only around 350 cases have been documented in the scientific literature so far. Case presentation We report a case of a 40-year-old North Indian, unmarried woman with a swelling on the left labium majora for 7 years, along with surface ulceration over the mass. Local examination showed a well-circumscribed, 8 × 8 cm pedunculated mass arising from the left labium majora with an overlying ulcer measuring 6 cm × 4 cm. After taking informed written consent, wide local excision of the mass and surrounding margins was done under anesthesia. Histopathology was suggestive of aggressive angiomyxoma. Immunohistochemistry was done, which was positive for estrogen and progesterone receptors. Her postoperative recovery was uneventful. The patient was given three doses of gonadotropin-releasing hormone agonist (injection, leuprolide 3.75 mg) once a month. No recurrence has been reported so far on follow-up visits for 1 year. Conclusion Aggressive angiomyxoma is one of the differential diagnoses for vulvovaginal growth in a female. As the tumor is well known for local recurrences, correct diagnosis and appropriate management using a multidisciplinary approach are crucial to managing such patients.

A deep giant aggressive angiomyxoma of the labia majora: A case report

“…Likewise, tumor recurrences are often asymptomatic. Other reported symptoms include dyspareunia, dysuria, pain, or discomfort related to the pressure effect of the mass on adjoining organs [7] .…”

Clinicopathological characteristics and prognosis analysis of Aggressive angiomyxoma: A Retrospective Study

“…It may be related to the hormone-responsive nature of AA, as its growth is under estrogen and progesterone in uence (12).…”

“…Until now, the exact mechanism of AA remains unclear. Nevertheless, since the tumor cells express desmin and smooth muscle actin, it is believed to originate either from specialized mesenchymal cells or from the multipotent perivascular progenitor cells (12). AA has the same molecular genetic background as other common mesenchymal tumors, with the translocation involving the chromosomal region 12q14-15 (13).…”

A Paraurethral Aggressive (Deep) Angiomyxoma

“…Aggressive angiomyxoma (AA) is a mesenchymal tumor that is histologically evident by its appearance of myxoid stroma with abundant vasculature [ 1 ]. The characterization of “aggressive” denotes the tumor's high tendency to invade locally and its propensity to infiltrate the perivaginal and perirectal tissue [ 2 ]. Aggressive angiomyxoma is six times more prevalent in females of reproductive age than in males, with a peak incidence in the third to fifth decades of life [ 2 ].…”

“…The characterization of “aggressive” denotes the tumor's high tendency to invade locally and its propensity to infiltrate the perivaginal and perirectal tissue [ 2 ]. Aggressive angiomyxoma is six times more prevalent in females of reproductive age than in males, with a peak incidence in the third to fifth decades of life [ 2 ]. In most of the reported cases, AA was observed in the pelvis or vulvar region.…”