Unmasking a Rare Pelvic Masquerader: Aggressive Vulvovaginal Angiomyxoma With Perineal Herniation

Aggressive angiomyxoma (AAM) is a rare, locally invasive, slow-growing low-grade mesenchymal tumor most frequently found in reproductive-age females with a high propensity for recurrence. AAM poses diagnostic challenges due to its indolent course, non-specific imaging appearance, and frequent misdiagnosis as more common entities like Bartholin cyst, lipoma, hernia, leiomyoma, etc. Accurate diagnosis before surgery is crucial to prevent recurrence. We present a case of AAM in a 47-year-old female with perineal herniation showing typical imaging features, including T2 swirling or laminated pattern, and correlated with histopathology post-surgical resection. The patient remained recurrence-free 12 months post-resection. This case reinforces the pivotal role of imaging, especially MRI, in preoperative diagnosis and surgical planning to minimize the likelihood of incomplete resection and recurrence.