Aggressive Angiomyxoma

Abstract

Aggressive angiomyxoma (AAM) is a rare soft tissue tumor typically in the pelvis and perineum in women of reproductive age, which is easily misdiagnosed. We describe a woman with vulvar AAM, initially mismanaged as a Bartholin cyst. However, a huge pelvic mass is noted on the following imaging studies. The characteristics of AAM on computed tomography and magnetic resonance imaging have been specified in the literature, but we further point out the potential value of sonography in diagnosing AAM. Besides, excisional biopsy may cause tumor bleeding in a case of AAM.

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

“…A total of 74 articles were included ( 4 – 77 ), containing 87 cases. Among the 74 studies, 37 were in Chinese ( 4 – 6 , 8 – 12 , 14 , 15 , 17 , 19 , 21 , 22 , 25 – 31 , 33 – 35 , 39 , 40 , 43 , 45 , 46 , 51 – 53 , 55 , 56 , 60 , 61 , 77 ), 34 were in English ( 7 , 13 , 16 , 18 , 20 , 23 , 24 , 32 , 36 , 38 , 41 , 42 , 44 , 47 , 50 , 54 , 57 – 59 , 62 – 76 ), and three were in Spanish ( 48 , 49 , 54 ). Most patients came from Asia (77.0%), especially China (60.1%), a few from Europe (13.8%) and North America (7.0%), and only very few were from Africa (1.1%) and South America (1.1%) ( Supplementary Table ).…”

Application of ultrasound in aggressive angiomyxoma: Eight case reports and review of literature

“…An AAM case report in 2012 described the lesion as a large hypoechoic to isoechoic mass on transvaginal ultrasound examination, with internal septa of high echogenicity, and rich blood signals scattering throughout the tumor on colour Doppler sonography[ 30 ]. Another case in 2013 also presented as a hypoechoic and heterogeneous mass with well-defined margins, abundant internal vascularity, and peripheral blood vessels[ 31 ]. The swirled sign observed on both ultrasound and MRI has also been mentioned by a recent study[ 26 ].…”

Deep (aggressive) angiomyxoma of the vulva in a pregnant woman

“…Aggressive angiomyxoma is an uncommon mesenchymal tumour, which usually appears in the pelvic region of women in their reproductive age 1–7. Although benign, it is a locally infiltrative tumour with frequent recurrences 1–7.…”

“…Aggressive angiomyxoma is an uncommon mesenchymal tumour, which usually appears in the pelvic region of women in their reproductive age 1–7. Although benign, it is a locally infiltrative tumour with frequent recurrences 1–7. These tumours have oestrogen and progesterone receptors which explains their tendency to grow with pregnancy 1 3 7…”

“…The primary treatment is surgical, with total excision of the tumour 1–7. Adjuvant therapy with gonadotropin-releasing hormone analogues (GnRH agonists) has been suggested for residual disease and recurrence treatment 3–8…”