• Bo Ram Yu, Won Ku Choi, Dong Hyu Cho, and Na-Ri Lee.
• Department of Obstetrics and Gynecology, Jeonbuk National University Hospital, Jeonbuk National University Medical School, Jeonju, Republic of Korea.
• Medicine (Baltimore). 2025 Jan 24; 104 (4): e41287e41287.

RationaleAggressive angiomyxoma (AAM) is an exceptionally rare mesenchymal tumor that predominantly manifests in the female genital organs during the reproductive age. Its rarity alone makes it a fascinating subject for study. The diagnosis of AAM necessitates differentiation from other benign or mesenchymal tumors and can be confirmed through immunohistochemistry (IHC) staining. Surgical resection is the primary treatment, and adjuvant treatment can be used as hormonal therapy with gonadotropin-releasing hormone agonists, selective estrogen receptor modulators, and aromatase inhibitors.Patient ConcernsA 44-year-old premenopausal Korean woman presented with a growing perineal mass and frequent urination.DiagnosesHistopathological findings confirmed AAM, with IHC staining showing estrogen receptor, progesterone receptor, actin and desmin positivity, and CD34 and S100 negativity.InterventionsThe mass was excised transvaginally under general anesthesia.OutcomesThe patient showed no signs of recurrence 6 months postoperatively.LessonsAAM in the vagina is a rare tumor that requires differential diagnosis using IHC staining. Previously, we reviewed reported cases and confirmed the feasibility and effectiveness of surgery as the main treatment. This might reassure us about the potential successful treatment of AAM. Adjuvant hormonal therapy with gonadotropin-releasing hormone agonists, selective estrogen receptor modulators, and aromatase inhibitors can further reduce the risk of recurrence.Copyright © 2025 the Author(s). Published by Wolters Kluwer Health, Inc.

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