NB09方案治疗高危和极高危神经母细胞瘤近期疗效观察

Short term curative effect of NB09 protocol on high-risk and ultra-high-risk neuroblastoma

目的评价中国儿童神经母细胞瘤协作组09方案（NB09方案）治疗高危和极高危神经母细胞瘤的初步疗效。方法回顾性分析2009年1月—2013年1月天津医科大学肿瘤医院收治的高危和极高危神经母细胞瘤患儿的临床及随访资料。38例患儿纳入分析,其中男27例,女11例。高危组7例,极高危组31例。诊断时月龄19-160个月（中位就诊月龄36.5个月）。高危组：术前新辅助化疗A、B方案交替4-6周期后评估、手术。极高危组手术前后化疗,然后自体干细胞移植,瘤床放疗,停化疗后维甲酸治疗同高危组。结果治疗结束时CR 25例,PR 5例,SD 3例,PD 5例,其中死亡2例,治疗总有效率（CR＋PR＋SD）86.8%。至随访结束,全组无瘤生存15例,带瘤生存9例,死于肿瘤复发7例,死于进展7例,生存期6-52个月（中位生存期25.5个月）,其1、2、3年总生存率（OS）分别为91.7%、64.5%和57.3%。Kaplan-Meier生存曲线和Log-rank检验提示高危组与极高危组之间生存率差异无统计学意义（P=0.56）。结论 NB09方案治疗高危和极高危神经母细胞瘤的近期疗效初步肯定,值得进一步临床验证。

Objective To evaluate the clinical outcome of NB09（China Pediatric Neuroblastoma cooperative group 09）protocol on children with high-risk and ultra-high risk neuroblastoma. Methods The clinical and follow-up data of pa-tients who suffered from high-risk（n=7） and ultra-high risk（n=31） neuroblastomas and admitted in Tumor hospital of Tian-jin Medical University between January 2009 to January 2013 were retrospectively reviewed（27 boys and 11 girls）. The ageat diagnosis was 19-160 months（median age was 36.5 months）. In the high risk group, patients were evaluated and operatedafter 4 to 6 circles of neoadjuvant chemotherapy. In ultra-high risk group, patient received chemotherapy before and after op-eration, then autologous stem cell transplantation and tumor bed radiotherapy. After chemotherapy, retinoic acid treatmentwas given to patients in ultra high risk group as in high risk group. Results At the end of treatment, 25 patients achievedcomplete remission; 5 patients achieved partial remission; 3 patients were in stable disease; 5 patients were deteriorating intheir conditions which lead to 2 deaths. In total, the response rate reaches upto 86.8%. By the end of follow up, 15 patientshad a disease-free-survival, 9 patients survived with tumor, 7 died from recurrence and 7 died from deteriorating conditions.Survival time ranged from 6 to 52 months（median survival 25.5 months）. The 1-, 2- and 3-year overall survival were91.7%, 64.5% and 57.3% respectively. Kaplan-Meier curve and Log-rank test showed no statistical significance betweenhigh risk and ultra-high risk neuroblastomas. Conclusion The outcome of NB09 protocol for high risk and ultra-high riskneuroblastoma was preliminary affirmed. It is worthy of further clinical verification.