Limb Salvage by Repetitive Radical Surgery in Recurrently Spreading Myxofibrosarcoma: Case Report and Literature Review.

Introduction: We report the case of a 70-year-old athletic female who was advised to undergo leg amputation following an R1 resection at another institution. Due to the patient's insistence on limb preservation, she underwent 17 resections for recurrent tumors in the proximal lower leg and distal thigh, accompanied by reconstruction with two free flaps and adjuvant radiotherapy. Histopathological analysis revealed a widespread, recurrent, cell-poor myxofibrosarcoma with prominent vascular architecture, measuring 6 × 3.5 × 2 cm, and staged as cT2 N0 M0, G2.

Case Presentation: After 17 resections targeting the periphery of previously excised lesions, combined with soft tissue reconstruction and 60 gray (Gy) irradiation, the patient has remained recurrence-free for over 10 years. At 80 years old, she continues to lead an active lifestyle, regularly engaging in sports. This outcome partly contrasts with our systematic literature review, which found a recurrence rate of up to 60% for aggressive surgical strategies in myxofibrosarcoma of the extremities, often requiring multiple surgeries, including amputations.

Conclusions: Recurrent myxofibrosarcoma should be treated with radical surgical intervention, even in cases of multiple recurrences beyond the original tumor site. By employing free flap reconstruction combined with irradiation, limb preservation can be facilitated, offering patients the potential to maintain functionality and quality of life.