Pelvic Aggressive Angiomyxoma: Major Challenges in Diagnosis and Treatment

Aggressive pelvic angiomyxoma is a very rare mesenchymal tumor that is usually diagnosed in premenopausal female patients. The current mainly reported treatment is wide surgical excision. Other treatment options, such as radiotherapy and hormonal therapy, have been suggested as potential alternatives. A 61-year-old postmenopausal female patient presented with hematuria that led to the identification of a perirectal mass on abdominopelvic imaging. A 46-year-old female patient presented with a perineal mass of unknown etiology. Despite extensive investigations, the diagnosis could not be confirmed before surgical resection in both patients. Surgical excisions were performed and revealed the presence of an aggressive angiomyxoma with positive estrogen and progesterone tumoral receptors in both cases. Radiological and clinical recurrence was noted in one patient. Tumor regression was noted in this patient after treatment with a luteinizing hormone-releasing hormone (LHRH) agonist with long-term remission. The diagnosis of a perirectal aggressive angiomyxoma is an exceedingly rare event. Preoperative biopsy and pathological diagnosis are challenging and often yields poor results. Its slow growth and expression of hormonal receptors make noninvasive therapeutic strategies, such as radiotherapy, gonadotropin-releasing hormone agonists, or even watchful waiting, valid options in selected patients. Due to the lack of reported cases, the best treatment has yet to be elucidated.