Fibrosarcoma is a mesenchymal tumor constituted by malignant fibroblasts. Myxofibrosarcoma is one of the fibrosarcoma variants that mostly develops from dermal/subcutaneous tissues. The most common locations are the limbs, with rare occurrences in the chest, head, and neck. Since, to best of our knowledge, there is no such report in English literature, we hereby present a case of lung myxofibrosarcoma. A 47-year-old man who had chest pain for 4 months was admitted to our clinic. The chest X-ray revealed a homogeneous density in the left upper lung. His chest computed tomography (CT) scan showed a solid mass lesion of 52 x 58 mm in size at the apical segment of the left upper lobe. There was tumor invasion at the second and third ribs. CT-guided fine-needle tru-cut lung biopsy was performed. Histopathological evaluation result was high grade myxofibrosarcoma. Surgery was conducted. Myxofibrosarcoma is a surgically curable disease. However, local recurrences occur in 50% to 60% of the cases. Therefore, chemotherapy and/or radiotherapy is the suggested approach following surgery.

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