Castel Sánchez V, Melero Moreno C, García-Miguel García-Rosados P, Navajas Gutiérrez A, Ruiz Jiménez J I, Navarro Fos S, Garín Valle J C, Galbe Sada M
Unidades de Oncología Pediátrica del Hospital Infantil La Fe de Valencia.
An Esp Pediatr. 1997 Dec;47(6):584-90.
Our objective was to carry out a prospective multicenter study of neuroblastoma patients diagnosed between 0 and 12 months of age.
Diagnostic procedures included histology, catecholamine excretion, bone marrow cytology and MIBG-scan. Staging was evaluated according to the INSS classification. After 1992, Simada criteria were used and also N-myc amplification, DNA index and P-glycoprotein determinations in tumoral tissue. The surgical technique employed and complications derived from it were also evaluated. The patients were treated according to stage with multicenter Spanish protocols N-I-87 and N-II-92. Overall survival and event free survival were calculated by actuarial methods.
Between October 1987 and June 1992, a total of 140 infants less than one year of age were registered and diagnosed of neuroblastoma, representing 40% of all neuroblastoma cases. Median age was 0.3 years and 73% were less than 6 months of age at diagnosis. The most frequent stage was 1 (35%) followed by 4-S (20%). The frequency of unfavorable prognostic factors was the following: LDH (21%), NSE (14%), ferritin (18%), Shimada (7%), DNA (35%), NMA (3%), TrakA (23%), P-glycoprotein (19%). Surgery was performed in 133 children: total resection was reported in 94 and > 90% in another 22 cases. Complications attributed to surgery occurred in 12% of the cases. Chemotherapy was given in 73 cases and radiotherapy in 7. The five year total survival is 91% and the event free survival 88%. Survival by stages: Stage 1 = 91%, stage 2A = 88%, stage 2B = 100%, stage 3 = 84%, stage 4 = 56% and stage 4-S = 100%.
我们的目的是对0至12个月大时被诊断为神经母细胞瘤的患者进行一项前瞻性多中心研究。
诊断程序包括组织学、儿茶酚胺排泄、骨髓细胞学和间碘苄胍扫描。根据国际神经母细胞瘤分期系统（INSS）分类评估分期。1992年后，使用了岛田标准，还对肿瘤组织进行了N - myc扩增、DNA指数和P - 糖蛋白测定。还评估了所采用的手术技术及其引发的并发症。患者根据分期采用西班牙多中心方案N - I - 87和N - II - 92进行治疗。通过精算方法计算总生存率和无事件生存率。
1987年10月至1992年6月期间，共登记并诊断出140例1岁以下的神经母细胞瘤婴儿，占所有神经母细胞瘤病例的40%。中位年龄为0.3岁，73%的患者在诊断时小于6个月。最常见的分期是1期（35%），其次是4 - S期（20%）。不良预后因素的发生率如下：乳酸脱氢酶（LDH，21%）、神经元特异性烯醇化酶（NSE，14%）、铁蛋白（18%）、岛田（7%）、DNA（35%）、N - 乙酰转移酶（NMA，3%）、原肌球蛋白相关激酶A（TrakA，23%）、P - 糖蛋白（19%）。133名儿童接受了手术：94例报告为完全切除，另外22例的切除率> 90%。手术相关并发症发生在12%的病例中。73例患者接受了化疗，7例接受了放疗。五年总生存率为91%，无事件生存率为88%。各分期的生存率：1期 = 91%，2A期 = 88%，2B期 = 100%，3期 = 84%，4期 = 56%，4 - S期 = 100%。
1）大多数1岁以下婴儿的神经母细胞瘤病例在6个月前被诊断出来。2）对于这个年龄组，1期和4 - S期是最常观察到的。3）不良生物学因素比1岁以上儿童少见，且与播散性疾病（晚期）相关。4）除4期患者外，预后良好。1期和2期病例可仅通过手术治疗。化疗可能对3期患者有益。
