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Background Aggressive angiomyxoma (AAM) is a rare mesenchymal tumor predominantly affecting the pelvis and perineum of females. Due to its variable clinical presentation, it is often misinterpreted and treated as other pathologies like Bartholin gland cyst, vulvar abscess, and hernia. The tumor is locally infiltrative, making its complete surgical removal difficult.  Aim The aim of the study is to highlight and illustrate the imaging and pathological features of AAM.  Materials and Methods After obtaining clearance from the Institutional Ethics Committee, a retrospective study was conducted on patients with histopathological diagnosis of AAM, presenting to the dedicated sarcoma clinic between 2016 and 2019. The demographic, clinical, radiological, pathological, and treatment details were obtained from the institute's database. The magnetic resonance imaging (MRI) and computed tomography images were reviewed to assess different imaging parameters. Follow-up imaging was assessed for residual or recurrent mass in the pelvis or perineum and distant metastasis.  Results A total of four patients were included with an age range of 28 to 50 years. The baseline MRI of these four patients shows pelvic mass extending into the perineum, infiltrating along the surrounding fascial planes with the characteristic laminated appearance on T2-weighted image and gradual intense enhancement following contrast administration. All patients had residual disease post-surgery and were put on hormonal therapy.  Conclusion AAMs are locally aggressive, rarely metastasizing mesenchymal tumor that has a specific predilection for the perineum and pelvis of females. MRI features like laminated or striated appearance, post-contrast enhancement, and finger-like infiltrating projections should raise the suspicion of the diagnosis on baseline imaging.

Radio-pathological and Clinical Correlation of Aggressive Angiomyxoma: Experience of a Tertiary Care Cancer Hospital