Background/aim: Malignant triton tumor (MTT) is a rare, highly aggressive malignant nerve sheath tumor with rhabdomyoblastic differentiation. The overall 5-year survival rate is extremely low, and no standardized treatment exists. We report a case of MTT in the distal femur that was treated with surgery and chemotherapy.
Case report: A 16-year-old male was referred to our hospital due to severe pain during physical activity and difficulty in walking. He showed no café-au-lait spots and no family history of Neurofibromatosis-1. He underwent an incisional biopsy and was diagnosed with MTT of the distal femur. After administering preoperative chemotherapy using adriamycin, ifosfamide, and cisplatin, wide resection surgery was performed for MTT. Subsequently, postoperative chemotherapy using methotrexate, in addition to the agents mentioned above, was administered. One and a half years after the surgery, he can walk without pain, and there are no signs of local recurrence or metastasis.
Conclusion: The combined modality treatment, integrating chemotherapy and surgery, has successfully prevented local recurrence and accomplished favorable outcomes without lung metastasis.
Keywords: Malignant triton tumor; bone; chemotherapy; malignant peripheral nerve sheath tumor; neurofibromatosis-1; surgery.
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