Department of Orthopaedic Surgery, Osaka Metropolitan University Graduate School of Medicine, Osaka, Japan;
Department of Orthopaedic Surgery, Osaka Metropolitan University Graduate School of Medicine, Osaka, Japan.
In Vivo. 2024 Nov-Dec;38(6):3112-3116. doi: 10.21873/invivo.13796.
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.
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.
The combined modality treatment, integrating chemotherapy and surgery, has successfully prevented local recurrence and accomplished favorable outcomes without lung metastasis.
背景/目的：恶性蝾螈瘤（MTT）是一种罕见的、高度侵袭性的神经鞘恶性肿瘤，具有横纹肌样分化。总体 5 年生存率极低，目前尚无标准化的治疗方法。我们报告了一例发生在股骨远端的 MTT 病例，该病例采用手术和化疗进行治疗。
一名 16 岁男性因运动时剧烈疼痛和行走困难而被转诊至我院。他没有牛奶咖啡斑，也没有家族性神经纤维瘤病 1 病史。他接受了切开活检，被诊断为股骨远端 MTT。在使用阿霉素、异环磷酰胺和顺铂进行术前化疗后，我们对 MTT 进行了广泛的切除手术。随后，在术后使用甲氨蝶呤，联合上述药物进行化疗。手术后 1 年半，他可以无痛行走，没有局部复发或转移的迹象。
化疗和手术相结合的综合治疗方法成功地预防了局部复发，并取得了良好的结果，没有发生肺转移。
