Objctive: To study the clinical effect of surgery combined with chemotherapy and radiotherapy in children with central primitive neuroectodermal tumor (cPNET), as well as the risks factors for poor prognosis.
Methods: A retrospective analysis was performed for the clinical data of 42 children who were diagnosed with cPNET from June 2012 to September 2018.
Results: The 42 children had a median overall survival (OS) time of 2.0 years and a median event-free survival (EFS) time of 1.3 years; the 1-, 3-, and 5-year OS rates were 76.2%±6.6%, 41.4%±8.7%, 37.3%±8.8% respectively, and the 1-, 3-, and 5-year EFS rates were 64.3%±7.4%, 32.7%±8.0%, 28.0%±8.1% respectively. The univariate analysis showed that there were significant differences in the OS and EFS rates among the children with different patterns of surgical resection, chemotherapy cycles, and risk grades (P<0.05), and there was also a significant difference in the OS rate between the children receiving radiotherapy and those not receiving radiotherapy (P<0.05). The multivariate Cox regression analysis showed that chemotherapy cycles and risk grade were independent influencing factors for EFS and OS rates (P<0.05). The EFS and OS rates increased with the increase in chemotherapy cycles and the reduction in risk grade.
Conclusions: Multimodality therapy with surgery, chemotherapy, and radiotherapy is an effective method for the treatment of cPNET in children. Early diagnosis and treatment and adherence to chemotherapy for as long as possible may improve EFS and OS rates.
目的: 分析手术联合化疗及放疗治疗儿童中枢性原始神经外胚瘤（cPNET）的疗效，并对其预后危险因素进行探讨。
方法: 收集2012年6月至2018年9月诊断为cPNET的42例患儿的临床资料并进行回顾性分析。
结果: 42例患儿的中位总生存（OS）期为2.0年，中位无事件生存（EFS）期为1.3年；1、3、5年OS率分别为76.2%±6.6%、41.4%±8.7%、37.3%±8.8%，1、3、5年EFS率分别为64.3%±7.4%、32.7%±8.0%、28.0%±8.1%。单因素分析结果显示，不同手术切除方式、化疗次数、危险度分级患儿OS率、EFS率比较差异均有统计学意义（P < 0.05），是否放疗患儿的OS率比较差异亦有统计学意义（P < 0.05）。多因素Cox回归分析结果显示，化疗次数、危险度分级是cPNET患儿OS率及EFS率的独立影响因素（P < 0.05），化疗次数越多，危险度越低，EFS率及OS率越高。
结论: 手术、化疗及放疗联合的综合治疗是目前治疗儿童cPNET的有效方法，早期发现早期治疗及尽量延长、坚持化疗有助于提高EFS率及OS率。