BACKGROUND:

Aggressive deep perineal angiomyxoma is a mesenchymal tumor of very limited occurrence that originates due to a tumorous growth of connective tissue that expands despite its benign nature; it is characterized by an aggressive behavior.

CLINICAL CASE:

38-year-old patient with a 3 cm genital nodule on the left labium majus, with clinical suspicion of Bartholin's gland cyst. During the intervention for its drainage, a soft, mesenchymal, pedunculated tumor was observed. Histological analysis confirmed that it was an aggressive deep perineal angiomyxoma. Following a negative extension study, a simple left vulvectomy was completed, with a favorable postoperative course.

CONCLUSIONS:

Obtaining an initial biopsy is critical, especially in cases of aggressive mesenchymal tumors, such as deep perineal aggressive angiomyxoma. In this way an individual treatment plan can be established based on the definitive histologic diagnosis.

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