Extraskeletal osteosarcoma is a rare soft tissue tumor. We report an exceptional case located in the forearm. A 62-year-old woman consulted for a tumor of the right forearm which she had noticed for six months. Physical examination revealed a 10 x 12 cm tumor with an ulcerated center. MRI demonstrated a heterogeneous mass exhibiting no connection with the bone or subjacent periosteum. Wide surgical resection was performed. The pathology study of the operative specimen confirmed the diagnosis of soft tissue osteosarcoma. The patient was given postoperative chemotherapy and was free of local recurrence or metastasis eighteen months after surgery. We discuss the present case and review data reported in the literature.
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