摘要目的 回顾分析Ⅳ期高危神经母细胞瘤(HR-NB)患儿放疗疗效、安全性及影响因素.方法 2014-2017间在北京儿童医院肿瘤内科按照BCH-HR-NB-2007方案治疗并行局部放疗的Ⅳ期HR-NB患儿120例中男56例、女64例,中位年龄43(9～ 148)个月.治疗方案为4疗程CAV方案化疗,3疗程CVP方案化疗,第4疗程后行手术切除,7疗程后予自体造血干细胞移植及局部15.0～30.6 Gy放疗(仅原发瘤灶放疗82例、原发瘤灶+转移灶放疗38例),后予13顺式-维甲酸维持治疗,总疗程约1.5年.结果 随访中位时间21个月.3年局部控制率为84.4％,3年无事件生存率放疗前转移灶无残留组高于有残留组(78.4％:30.4％,P=0.003),放疗距瘤灶切除时间＜6个月组高于≥6个月组(66.1％:50.6％,P=0.018).转移灶有残留患儿未行、行转移灶放疗的进展率分别为66.6％、20.0％ (P=0.001).所有患儿均未发生肝脏、肾脏、心脏等放疗相关不良反应,3-4级骨髓抑制率放疗后1周为24.5％,2周为8％.结论 放疗对于Ⅳ期HR-NB患儿局部控制存在确切疗效,术后尽早放疗及对病初转移灶进行放疗有助于进一步改善预后.短期观察未见重要脏器损害,放疗后2周骨髓抑制呈恢复趋势.

abstractsObjective To retrospectively analyze the clinical efficacy,safety and influencing factors of radiotherapy in children with stage Ⅳ high-risk neuroblastoma (HR-NB).Methods A total of 120 children with HR-NB who were diagnosed and treated with local radiotherapy according to the BCH-HR-NB-2007 protocol in the Oncology Department of Beijing Children's Hospital from January 2014 to December 2017 were enrolled.Among them,56 children were male and 64 female with a median age of 43 months (9 -148 months).The treatment protocol consisted of 4 cycles of CAV chemotherapy,3 cycles of CVP chemotherapy,surgical resection after 4 cycles,autologous hematopoietic stem cell transplantation after 7 cycles,local radiotherapy at a dose of 15.0-30.6 Gy for 82 cases of primary tumors and 38 cases of primary and metastatic tumors,followed by 13 cis-retinoic acid as maintenance therapy.The entire treatment protocol endured for approximately 18 months.Results The median follow-up time was 21 months.The 3-year local control rate was 84.4％.Before radiotherapy,the 3-year event-free survival rate was 78.4％ in children without metastases,significantly higher compared with 30.4％ in the residual group (P=0.003).The 3-year event-free survival rate was 66.1％ in patients who underwent radiotherapy within 6 months after surgery,significantly higher than 50.6％ in their counterparts receiving radiotherapy at 6 months or more after surgery (P=0.018).Among the children with residual metastases before radiotherapy,the progression rate in children who did not receive radiotherapy was 66.6％,significantly higher compared with 20.0％ in those receiving radiotherapy (P=0.001).All patients had no radiation-related adverse reactions in the liver,kidney and heart,etc.The incidence rate of grade Ⅲ-Ⅳ myelosupression was 24.5％ at 1 week post-radiotherapy,and 8％ at 2 weeks after radiotherapy.Conclusions Radiotherapy yields definite clinical efficacy in the local control of children with stage Ⅳ HR-NB.Early radiotherapy after surgery and radiotherapy for the metastatic lesions can improve the clinical prognosis.No vital organ injuries are observed during the short-term follow-up.At 2 weeks after radiotherapy,the myelosupression is gradually restored.