Aggressive Pelvic Angiomyxoma Presenting As Vulvar Swelling in a Young Female: A Radiologic and Pathologic Correlation

Aggressive angiomyxoma (AA) is a rare mesenchymal tumor that primarily occurs in women of reproductive age and arises in the pelvis and perineum (perineal area). We present the case of a 20-year-old unmarried female from Afghanistan who was sent for assessment of worsening swelling in the left vulvar and perineal regions. Imaging demonstrated a large, minimal enhancement, soft tissue mass in the left hemipelvis, which displaced organs but otherwise demonstrated no invasion. The lesion extended to the ischiorectal fossa and perineum. Owing to the clinical and radiologic findings, aggressive angiomyxoma was provisionally diagnosed, which was confirmed histologically by image-guided biopsy. The patient underwent surgical resection. Given the tumor has a known local recurrence risk, and it stained positive for estrogen and progesterone receptors, long-term follow-up and adjuvant hormonal treatment were recommended. This case highlights the importance of considering aggressive angiomyxoma in the imaging workup of vulvoperineal masses in younger women and the importance of multidisciplinary management, including imaging, to optimize patient outcomes.