Recurrent Aggressive Angiomyxoma: A Single-Institution Case Series on Management and Outcomes

Aggressive angiomyxoma (AA) is a rare, locally infiltrative mesenchymal tumor of the pelviperineal region that carries a significant management challenge due to its high rate of local recurrence. While histologically benign, its clinical behavior can be unpredictable. Optimal strategies for managing recurrent disease and the full biological potential of AA are not well defined. Case Presentation: We present two cases of recurrent AA managed at our institution. First case involved a 31-year-old woman with a margin-positive resection of a pelvic recurrence, initially misdiagnosed as a capillary hemangioma, who showed partial tumor regression with adjuvant tamoxifen and radiotherapy. Second case describes a 33-year-old woman with a large, recurrent pelvic tumor successfully managed with resection and adjuvant tamoxifen. Conclusion: This series identifies several key clinical lessons. First, AA can mimic other benign lesions, emphasizing the need for expert pathological and radiological review. Second, while complete surgical excision is the goal, adjuvant therapy may play a role in managing residual or recurrent disease. Lastly, as late recurrence is well documented, lifelong imaging surveillance remains crucial even after successful treatment.