Beatriz Fevereiro, Ricardo Fonseca, Teresa Margarida Cunha
Deep (aggressive) angiomyxoma is a rare mesenchymal tumor that primarily affects premenopausal women and is frequently misdiagnosed because of its deep pelvic location, indolent growth, and nonspecific clinical presentation. It is typically multicompartmental and demonstrates a characteristic laminated ("swirled") appearance on T2-weighted magnetic resonance imaging (MRI), reflecting alternating myxoid and fibrous stromal components. Local recurrence is common after surgical resection, making long-term imaging surveillance essential. Tumor expression of estrogen and progesterone receptors provides a biological rationale for hormonal therapy. We report a case of recurrent pelvic deep (aggressive) angiomyxoma in a 45-year-old woman who achieved complete and sustained radiologic remission documented by serial MRI throughout 8 years of continuous gonadotropin-releasing hormone (GnRH) agonist therapy. This uncommon long-term outcome highlights both the potential for durable disease control with hormonal therapy in deep (aggressive) angiomyxoma and the value of MRI for longitudinal assessment of treatment response.