Aggressive angiomyxoma of the pelvis and perineum: a case report and review of the literature

Aggressive angiomyxoma is a rare mesenchymal tumour arising from soft tissue of the pelvis and perineum. Other features of this tumour are non-specific clinical signs, local invasion, a high local recurrence rate, and recurrence long after initial excision. We provide a case report with a review of the literature. A 57-year-old woman with a large bulky mass in the perineum was admitted to our institution. She had previously undergone iterative surgery for excision of pelvic and perineal masses. The preoperative diagnostic procedure is described as well as the therapeutic surgical approach (a double simultaneous laparotomic and perineal approach was opted for). The surgical procedure was difficult, time-consuming and dangerous owing to an extensive area of hard retroperitoneal sclerosis involving the low urinary viscera, the vaginal stump and the mesorectum. Radical excision of the mass favoured the re-establishment of the normal pelvic anatomy, resolution of the preoperative symptoms and complete recovery of working capability. Surgical excision is the gold standard in the treatment of aggressive angiomyxoma. This benign neoplasm may sometimes present a malignant course owing to involvement of pelvic viscera. It is also associated with a high late recurrence rate due to local aggressiveness, and longterm follow-up is therefore necessary.