Second Recurrence of Aggressive Angiomyxoma of Labia Majora in a 34-Year-Old Woman: A Case Report and Review of Literature

Abstract Aggressive angiomyxoma (AAM) is a rare, slow-growing, benign neoplasm with high recurrence and local invasion. It is usually asymptomatic and frequently presents as a mass affecting the perineal and pelvic regions of women in reproductive age group. We present a rare case of a 34-year-old woman with second recurrence of a giant AAM arising from labia majora. The patient presented with a slow-growing pedunculated mass (around 20 × 12 cm) over the right labia majora for the past 1 year. In the last 10 years, she was operated on two different occasions (2013 and 2015) for similar lesion and was a confirmed case of AAM. Ultrasound of the lesion and magnetic resonance imaging of the abdominopelvic region was suggestive of recurrent AAM. The patient underwent en bloc dissection of the tumor with negative margin. Histopathological examination confirmed the diagnosis of recurrent AAM. En bloc dissection with negative margin leads to complete removal of tumor mass. However, long-term follow-up with annual magnetic resonance imaging is advised.