Myxofibrosarcoma or myxoid malignant fibrous histiocytoma is one of the most common sarcomas of the limb. It is usually treated multimodally. Most frequent sites of metastasis are the bone, lung and lymph nodes. The present paper is a case report of a 65-year-old male with myxofibrosarcoma of the fibularis longus muscle, for which he first underwent surgery - tumor resection with appropriate margins. The tumor was staged pT2b cN0 cM0. Postoperative PET-CT revealed metabolically inactive pulmonary nodules. Two months after surgery, he underwent adjuvant radiotherapy, a total dose of 60 Gy and 6 courses of chemotherapy (doxorubicin and ifosfamide). Pulmonary nodules have been stationary on all subsequent imagistic studies. He is free of recurrence on long-term follow-up.
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