Vulvar aggressive angiomyxoma: a surgical challenge

Abstract

Aggressive angiomyxoma is a rare and locally aggressive mesenchymal tumour, predominantly occurring in women of reproductive age group. The term aggressive is attributed to the infiltrative nature and frequent local recurrences. They arise commonly from the vulvovaginal region, perineum or pelvis and are usually misdiagnosed as other common entities in these regions. Radiological investigations aid in the diagnosis and planning of surgery. However, the final diagnosis in most of the cases is established by histopathological examination. We herein report a case of a middle-aged woman presenting with recurrent large right vulvar mass highlighting the surgical challenges posed by its intrapelvic extension.

Characteristics and treatment strategies of aggressive angiomyxoma in women: A retrospective review of 87 cases

“…Among the first-onset cases (65 cases), only seven patients were initially diagnosed with AAM, for which different auxiliary examinations were required. Three patients underwent needle biopsy ( 61 , 68 , 69 ), four MRI ( 51 , 60 , 61 , 68 ), and one ultrasound ( 53 ). For patients with incorrect initial diagnosis, bartholin's cyst or abscess and lipoma were the common conditions considered in the preliminary diagnosis .…”

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

“…This showed no statistically signi cant differences. In recent years, numerous studies have shown that incomplete or partial resection is acceptable, especially when high operative morbidity is anticipated and preservation of fertility is an issue (7,(18)(19)(20). The combination of incomplete resection with adjuvant therapy may be an effective treatment for AA, including hormonal therapy, arterial embolization, or radiation therapy.…”

“…Currently, Diana recommends that the evaluation of hormonal therapy as an adjunct to surgery is necessary (23). Other treatment modalities like angiographic embolization can be used in adjuvant setting and in cases where surgery is a contraindication or the possibility of obtaining a negative margin is dismal during resection (7). Furthermore, angiographic embolization as an alternative approach is not typically used solely and is often used in combination with other treatments, as the tumors usually being supplied with numerous feeding vessels (19).…”

“…Regarding these facts, hormonal therapies have been used for management of AA. There have been many successful cases in which estrogen and progesterone receptor-positive AA tend to respond to therapy with raloxifene, tamoxifen, and gonadotropin-releasing hormone (GnRH) agonist, such as leuprolide (7,21). FUCÀ et al con rmed that hormone therapy is an active treatment option, with a potential of disease control and of being combined with surgery (22).…”

“…However, some evidence exists that incomplete or partial resection is acceptable, especially when high operative morbidity is anticipated and preservation of fertility is an issue (4). AA may bene t from hormonal therapy such as GnRH agonists (6,7). Radiation therapy has also achieved some results in the treatment of AA (8,9).…”