Aggressive angiomyxoma is a distinctive, locally aggressive, mesenchymal tumor that preferentially involves the pelvic and perineal regions of females with a peak incidence in the 4 th decade of life. The neoplastic cells of aggressive angiomyxoma exhibit fibroblastic and myofibroblastic features and appear to be hormonally influenced. There is a strong propensity for local recurrence. The recurrence may be seen years after the initial resection; hence, long-term follow-up is required. We report a case of a 22-year-old woman with a huge pedunculated aggressive angiomyxoma arising from right labia majora.
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