【摘要】 [目的]探讨影响神经母细胞瘤(neuroblastoma,NB)患儿预后的因素。[方法]用回顾性研究方法对在日本兵库县立儿童医院随访资料完整的60例NB患儿,按首次就诊时临床资料(年龄、性别、原发部位、转移部位、Shimada组织学分型、INSS分期、手术能否根治性切除);生化指标(尿香草杏仁酸/高香草酸(vanillylmandelic acid/homovanillic acid,VMA/HVA比值)、血清乳酸脱氢酶(lactate dehydrogenase,LDH)、血清铁蛋白(ferritin)、神经原特异性烯醇化酶(neuron specific enolase,NSE)、肿瘤组织 NMYC拷贝及 DNA指数)等因素进行分组。以 Kaplan-Meier法计算无事件生存率(event-free survival,EFS)。单因素分析用Kaplan-Meiter生存曲线法比较组间EFS,显著性检验采用Log-rank法。多因素分析用COX模型处理各组数据。[结果]单因素分析发现:年龄>1岁、腹部原发、骨骼转移、INSS第3期,4期、Shimada组织类型不良型、无法根治性切除、血清LDH>1500 IU/L、ferritin>150μg/L、NSE>100μg/L、肿瘤组织 NMYC拷贝>l及 DNA指数=1等组别的5年EFS均显著低于其对照组。性别与尿 HVA/VMA比值对EFS无影响。COX模型多因素分析显示:只有骨骼转移、Shimada组织类型不良与NMYC拷贝>1对EFS产生不良影响。[结论]早期行肿瘤组织原癌基因NMYC分析,S更多还原

【Abstract】 [Objective] To investigate variables that contribute to the prognosis of neuroblastoma. [Methods] Data of 60 neuroblastoma patients who were continuously followed up in Kobe Children’s Hospital were studied retrospectively. Kaplan-Meier methods were used to estimate the event-free sur-vival(EFS) from the time of diagnosis. For univariate analysis, the Kaplan-Meier survival was used to compare the EFS probabilities; Log-rank statistic was used to test the significance of the differences in 5-year EFS between subgroups of patients. Analysis of multivariables was performed using the Cox regression method. The examined factors including clinical variables (age,sex, primary site of turnor metastatic sites, Shimada classification, stages,surgical resection) and biological variables(urine HVA/ VMA ratio、Kserum LDH、ferritin、NSE、NMYC gene amplification and DNA index of tumor tissue). [Results] Univariate analysis showed that five-year EFS of patients with age>1 yea, abdominal origin bone metastasis、 INSS 3-4 stage、Shimada unfavorable histology、unresectable tumor、or with serum LDH>1 500 IU/U ferritin>150μg/L NSE>100μg/L、or with NMYC gene amplification and DNA index = 1 was significantly lower than that of the control group. Multivariate analysis demonstrated that only the bone metastasis, NMYC gene amplification and unfavorable Shimada his- tology contributed negative effect to the EPS of neuroblastoma. [Conclusion] Early detection of NMYC gene amplification, DNA index of tumor tissue and bone scanning are important for evaluation of outcome of neuroblastoma.更多还原