Abstract
BACKGROUND: Aggressive angiomyxoma is a rare mesenchymal tumour of the genital tract with a high incidence in women of reproductive age. The aim of our work is to understand which is the best management strategy for this condition, starting from the description of a rare case report up to the performance of a narrative review of the literature. METHODS: We report a case of a 46-year-old woman who came to our attention because of the growth of a 10-centimetre pedunculated, non-soft, non-tender mass of the left labium majus. She underwent surgical excision, and the histologic diagnosis was aggressive angiomyxoma. Due to a lack of tumour-free margins, radicalization surgery was carried out after three months. A review of the literature of the last ten years was performed following the PRISMA statement on MEDLINE (PubMed). We obtained data from twenty-five studies describing thirty-three cases. RESULTS: Aggressive angiomyxoma is characterized by a high recurrence rate of between 36 and 72% after surgery. There is no universal consensus about hormonal therapy, and most studies (85%) describe surgical excision followed by only clinical and radiological follow-up. CONCLUSION: Wide surgical excision is the gold-standard treatment for aggressive angiomyxoma, succeeded by either clinical or radiological (ultrasound or MRI) follow-up.