Aggressive Angiomyxoma of the Vulva: Case Report and Literature Review

Abstract

Aggressive angiomyxoma is a rare soft-tissue tumour, typically occurring in the female pelvis and carrying a high risk of local infiltration and relapse. Surgery remains the first line of treatment, however some adjuvant treatments, such as gonadotrophin-releasing hormone (GnRH) agonists, have been used for primary treatment and for treatment against tumour recurrence. We describe a case of vulvar aggressive angiomyxoma in a 31-year old woman who underwent surgical excision of the tumour. Diagnosis was made on the basis of histopathological examination and positive immunohistochemical staining with oestrogen and progesterone receptors. Postsurgery, a GnRH agonist (3.75 mg triptorelin) was injected intramuscularly every month for 3 months to prevent tumour recurrence and, to date, no relapse has been observed. Whether treatment is with surgery, hormone therapy or both, it is clear that aggressive angiomyxoma requires close, long-term follow-up to monitor for disease recurrence and that the individualization of each case is essential for adequate management.