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

The International Neuroblastoma Risk Group (INRG) Staging System: An INRG Task Force Report
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DOI:
10.1200/jco.2008.16.6876
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发表时间:
2009-01-10
影响因子:
45.3
通讯作者:
Pearson, Andrew D. J.
Pearson, Andrew D. J.
中科院分区:
医学1区
文献类型:
--
作者:
Monclair, Tom;Brodeur, Garrett M.;Pearson, Andrew D. J.

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目的国际神经母细胞瘤危险组(INRG)分类系统的发展是为了建立一个共识的方法,治疗前的危险分层。由于国际神经母细胞瘤分期系统(INSS)是一个手术后分期系统,一个新的临床分期系统需要为INRG预处理风险classification system.MethodsTo阶段患者在任何治疗前,INRG工作组,由来自澳大利亚/新西兰,中国,欧洲,日本和北美的神经母细胞瘤专家组成,基于临床标准和影像学定义的风险因素(IDRF)开发了一种新的INRG分期系统(INRGSS)。为了探讨IDRFs对预后的影响,生存分析进行了661欧洲患者与INSS阶段1,2,或3疾病的IDRFs是knowed.ResultsIn的INGRSS,局部肿瘤分期L1或L2的基础上的一个或多个20 IDRFs,分别存在或不存在。转移性肿瘤定义为M期,MS期除外,其中转移局限于年龄小于18个月的儿童的皮肤、肝脏和/或骨髓。在661例患者队列中,21%的1期患者、45%的2期患者和94%的3期患者存在IDRF(即L2期)。INRGSS L2期疾病患者的5年无事件生存率显著低于INRGSS L1期疾病患者(78% +/-4%v 90% +/- 3%; P = 0.0010)。结论使用神经母细胞瘤的新分期(INRGSS)和风险分类(INRG)将极大地促进在世界不同地区进行的基于风险的临床试验的比较。
PurposeThe International Neuroblastoma Risk Group (INRG) classification system was developed to establish a consensus approach for pretreatment risk stratification. Because the International Neuroblastoma Staging System (INSS) is a postsurgical staging system, a new clinical staging system was required for the INRG pretreatment risk classification system.MethodsTo stage patients before any treatment, the INRG Task Force, consisting of neuroblastoma experts from Australia/New Zealand, China, Europe, Japan, and North America, developed a new INRG staging system (INRGSS) based on clinical criteria and image-defined risk factors (IDRFs). To investigate the impact of IDRFs on outcome, survival analyses were performed on 661 European patients with INSS stages 1, 2, or 3 disease for whom IDRFs were known.ResultsIn the INGRSS, locoregional tumors are staged L1 or L2 based on the absence or presence of one or more of 20 IDRFs, respectively. Metastatic tumors are defined as stage M, except for stage MS, in which metastases are confined to the skin, liver, and/or bone marrow in children younger than 18 months of age. Within the 661-patient cohort, IDRFs were present (ie, stage L2) in 21% of patients with stage 1, 45% of patients with stage 2, and 94% of patients with stage 3 disease. Patients with INRGSS stage L2 disease had significantly lower 5-year event-free survival than those with INRGSS stage L1 disease (78% +/- 4% v 90% +/- 3%; P = .0010).ConclusionUse of the new staging (INRGSS) and risk classification (INRG) of neuroblastoma will greatly facilitate the comparison of risk-based clinical trials conducted in different regions of the world.