The International Neuroblastoma Risk Group (INRG) Classification System: An INRG Task Force Report

The International Neuroblastoma Risk Group (INRG) Classification System: An INRG Task Force Report
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DOI:
10.1200/jco.2008.16.6785
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发表时间:
2009-01-10
影响因子:
45.3
通讯作者:
Matthay, Katherine K.
Matthay, Katherine K.
中科院分区:
医学1区
文献类型:
--
作者:
Cohn, Susan L.;Pearson, Andrew D. J.;Matthay, Katherine K.

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由于目前对神经母细胞瘤(NB)儿童的风险分类和治疗分层的方法在世界各地差异很大,因此很难直接比较基于风险的临床试验。建立了国际神经母细胞瘤风险组(INRG)分类系统,以建立治疗前风险分层的共识方法。患者和方法分析了1990年至2002年北美和澳大利亚(儿童肿瘤组)、欧洲(国际儿科肿瘤学会欧洲神经母细胞瘤组和德国儿童肿瘤和血液学组)和日本的8,800名被诊断为NB的儿童的13个潜在预后因素的统计和临床意义。结果年龄、组织学类型、肿瘤分化程度、MYCN癌基因状态、染色体11q状态和DNA倍体是最具统计学意义和临床意义的因素。一种基于临床标准和肿瘤影像的新的分期系统(INRG分期系统)被开发用于INRG分类系统。最佳年龄界限被确定为15至19个月,18个月被选为分类系统。根据临床标准和按INRG标准分层的队列中统计学上显著不同的EFS,定义了16个预治疗组。5年EFS超过85%的患者,超过75%到=50%到
PurposeBecause current approaches to risk classification and treatment stratification for children with neuroblastoma (NB) vary greatly throughout the world, it is difficult to directly compare risk-based clinical trials. The International Neuroblastoma Risk Group (INRG) classification system was developed to establish a consensus approach for pretreatment risk stratification.Patients and MethodsThe statistical and clinical significance of 13 potential prognostic factors were analyzed in a cohort of 8,800 children diagnosed with NB between 1990 and 2002 from North America and Australia (Children's Oncology Group), Europe (International Society of Pediatric Oncology Europe Neuroblastoma Group and German Pediatric Oncology and Hematology Group), and Japan. Survival tree regression analyses using event-free survival (EFS) as the primary end point were performed to test the prognostic significance of the 13 factors.ResultsStage, age, histologic category, grade of tumor differentiation, the status of the MYCN oncogene, chromosome 11q status, and DNA ploidy were the most highly statistically significant and clinically relevant factors. A new staging system (INRG Staging System) based on clinical criteria and tumor imaging was developed for the INRG Classification System. The optimal age cutoff was determined to be between 15 and 19 months, and 18 months was selected for the classification system. Sixteen pretreatment groups were defined on the basis of clinical criteria and statistically significantly different EFS of the cohort stratified by the INRG criteria. Patients with 5-year EFS more than 85%, more than 75% to = 50% to