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Title: SECOND RECURRENCE OF AGGRESSIVE ANGIOMYXOMA OF LABIA MAJORA : A CASE REPORT
e-poster Number: EP 195
Category: Miscellaneous
Author Name: Dr. Pratima Devram Rathod
Institute: Government Medical College Nagpur
Co-Author Name:
Abstract :
Introduction :Aggressive angiomyxoma (AAM) is a rare, slow-growing benign tumor, mainly affecting women. It typically involves the perineal and pelvic regions, with a moderate-to-high recurrence risk. We present a case of second recurrence from the labia majora. Case Description: A 34-year-old female presented with a slowly growing giant swelling on the right labia majora over the past year, causing perineal heaviness and abstinence. She had two prior surgeries for similar lesions in 2013 and 2015, with histopathology confirming aggressive angiomyxoma (AAM). Local examination revealed a soft, nontender, pedunculated mass (20x12 cm). Ultrasound showed a well-defined, hyperechoic lesion (12.2x12.1x5 cm) in the right labia majora. MRI revealed a hyperintense, well-defined lesion (19x10x8.7 cm) without adjacent infiltration. Case Discussion: Aggressive angiomyxoma (AAM) is a rare, benign tumor primarily affecting women of reproductive age, with the highest incidence between 20 to 50 years. It commonly involves the perineum, vulva, vagina, and pelvic regions. Though benign, AAM has a high recurrence rate (35-72%) due to its tendency for local tissue infiltration. The tumor cells are estrogen and progesterone receptor-positive, often growing during pregnancy. Imaging techniques like ultrasound and MRI are essential for diagnosis and monitoring recurrence. Gonadotropin-releasing hormone agonists and ER/PR-targeted therapies may shrink or prevent recurrence. Complete surgical resection with negative margins is the primary treatment to reduce recurrence, with adjunct hormonal therapies sometimes used. While AAM is generally non-metastasizing, long-term follow-up is crucial due to high local recurrence rates and potential for rare metastasis. Conclusion: AAM is a benign and locally aggressive tumor. Physical examination and imaging studies help narrow down the differential diagnosis, but HPE is the confirmatory modality. En bloc surgical excision with negative margin is desired to prevent recurrence and a long-term annual follow-up with MRI is advised.