Abstract
INTRODUCTION AND IMPORTANCE: Aggressive Angiomyxoma (AA) is an uncommon, locally infiltrative mesenchymal tumor that primarily originates from perineal and pelvic sites of women, particularly in the 4th decade of life with having an emphasized tendency for local recurrence, whereas it has a low tendency to metastasize. Patients often present with nonspecific symptoms such as painless visible mass that might be misdiagnosed with every mass in genital and pelvic sites in reproductive-age women. CASE PRESENTATION: We describe a 31-year-old female presenting with an enlargement on the right labia majora. Ultrasound and magnetic resonance were carried out, and the mass was surgically excised completely and without complication. The diagnosis of AA was made based on characteristic histopathological features. The postoperative follow-up for recurrence is currently being continued. CLINICAL DISCUSSION: Due to its rarity and lack of specificity in clinical and radiological examinations, the pre-operative misdiagnosis rate of AA is rather high. Hence, most cases are diagnosed on histology after initial surgical excision. Surgical management is the gold standard treatment for primary tumors; however, in case of local recurrences, treatment choices range from surgical resection to gonadotropin-releasing hormone (GnRH) agonist for tumors positive for estrogen and progesterone receptors. CONCLUSION: Wide surgical resection is the gold standard treatment of AA; however, exceptions might occur due to the depth of tumor infiltration to adjacent viscera. Therefore, adjunct medical therapies can play a crucial role in treatment. In addition, long-term follow-up is necessary due to the high rate of local recurrences.