以达卡巴嗪为基础的一线治疗方案在晚期恶性周围神经鞘膜瘤中的探索与疗效分析

Exploration and efficacy analysis of first-line treatment based on dacarbazine in advanced malignant peripheral nerve sheath tumor

目的分析以达卡巴嗪为基础的一线治疗方案在晚期恶性周围神经鞘膜瘤(MPNST)中的疗效。方法收集2014年6月到2021年10月收治的5例晚期MPNST患者的临床及随访资料(男性4例,女性1例;发病年龄14~57岁;ECOG评分1~3分;散发型4例,NF1相关型1例)。5例患者均接受以达卡巴嗪为基础的一线化疗,其中1例患者探索化疗联合免疫检查点抑制剂,1例NF1相关型患者探索化疗联合mTOR通路抑制剂西罗莫司。2例患者在获得最佳疗效时行放疗或手术治疗。总结患者临床病例资料并分析疗效。结果5例患者均有不同程度临床症状的缓解;3例患者最佳疗效为部分缓解,2例患者最佳疗效为疾病稳定。无进展生存时间为5.6~41.9个月;总生存时间为20.9~82.6个月。不良反应以骨髓抑制及消化系统反应为主。结论以达卡巴嗪为基础的一线治疗方案在晚期MPNST患者中显示出良好的疗效。

AIM To analyze the efficacy of first-line treatment based on dacarbazine in advanced malignant peripheral nerve sheath tumors(MPNST).METHODS Clinical data of 5 advanced MPNST patients from June 2014 to October 2021 were collected(4 males and 1 female;the onset age was 14-57 years old;ECOG score was 1-3 points;4 sporadic MPNST and 1 NF1 MPNST).All 5 patients received dacarbazine based first-line chemotherapy,in which 1 patient was treated with chemotherapy combined with immune checkpoint inhibitor,and 1 NF1 MPNST patient was treated with chemotherapy combined with mTOR pathway inhibitor sirolimus.Two patients received radiotherapy or surgery at the best response.The clinical data and the efficacy were summarized and analyzed.RESULTS The relief of clinical symptoms were observed in all 5 patients.The best response was partial response in 3 patients,and stable disease in 2 patients.Progression-free survival was 5.6 to 41.9 months,and the overall survival was 20.9 to 82.6 months.The main adverse reactions of the treatment were myelosuppression and digestive system reaction.CONCLUSION The first-line treatment based on dacarbazine has shown good efficacy in patients with advanced MPNST.