Int J Surg Case Rep. 2025 Nov;136:112057. doi: 10.1016/j.ijscr.2025.112057. Epub 2025 Oct 15.

INTRODUCTION AND IMPORTANCE: Aggressive angiomyxoma is a rare, slow-growing mesenchymal tumor typically found in the vulvo-perineal and pelvic region of women of reproductive age. Its benign but locally invasive nature and high recurrence rate make accurate diagnosis and management challenging. This case highlights a common diagnostic pitfall and underscores the importance of imaging and histopathological confirmation.

PRESENTATION OF CASE: A 32-year-old woman presented to the emergency department with a painful vulvar mass initially diagnosed as bartholinitis. Magnetic resonance imaging (MRI) revealed a well-limited mass in the left labia majora extending to the clitoris. Initial surgical excision resulted in an R2 margin, confirmed by histopathology to be aggressive angiomyxoma. The patient underwent a mandatory surgical revision, achieving R0 excision. The postoperative course was uneventful, with a good outcome at the six-month follow-up.

CLINICAL DISCUSSION: This case illustrates the propensity for aggressive angiomyxoma to be misdiagnosed as more common conditions like Bartholin’s gland cysts. MRI is the imaging modality of choice for characterizing the lesion and planning surgery. Complete surgical excision (R0) is the cornerstone of treatment to minimize the high risk of local recurrence. Hormonal therapy with GnRH analogues can be considered as an adjuvant treatment.

CONCLUSION: Aggressive angiomyxoma is a rare pathology that requires a high index of suspicion. Management should be undertaken in specialized centers with expertise in soft tissue tumors to ensure complete resection and manage potential recurrences. Increased awareness among clinicians is crucial for early diagnosis and appropriate treatment.

PubMed:41332071 | DOI:10.1016/j.ijscr.2025.112057