RESUMO
Malignant peripheral nerve sheath tumors are soft tissue sarcomas originating from peripheral nerves. They are more frequently diagnosed in individuals with neurofibromatosis and tend to affect young men more often than women. The most common sites for these tumors within the peripheral nerve sheath are in the pelvis and the distal femur. Although chemotherapy and radiotherapy are not frequently used, it should be noted that in some cases, postoperative radiotherapy and chemotherapy may be beneficial. The primary treatment approach typically involves the complete surgical removal of the tumor. Here, we discuss the case of our patient whom we successfully treated with extensive resection and followed up with postoperative radiotherapy at our clinic.