Changes over three decades in outcome and the prognostic influence of age-at-diagnosis in young patients with neuroblastoma: A report from the International Neuroblastoma Risk Group Project
Changes over three decades in outcome and the prognostic influence of age-at-diagnosis in young patients with neuroblastoma: A report from the International Neuroblastoma Risk Group Project
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DOI：
10.1016/j.ejca.2010.10.022
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发表时间：
2011-03-01
影响因子：
8.4
通讯作者：
London, Wendy B.
中科院分区：
文献类型：
--
作者：
Moroz, Veronica;Machin, David;London, Wendy B.
目的：自20世纪70年代以来，年龄增加一直是神经母细胞瘤（NB）儿童的一个不利危险因素，治疗分层的诊断年龄为12个月。在过去的30年里，对0 - 12个月患有晚期疾病的儿童的治疗强度有所增加；为了研究该策略是否改善了预后和/或降低了年龄对预后的影响，我们分析了国际神经母细胞瘤风险组（INRG）数据库。患者和方法：数据来自澳大利亚、欧洲、日本和北美的11037例NB患儿（1974-2002）。无事件生存期（EFS）的Cox模型测试了年龄和诊断年龄的预后意义，调整了骨髓（BM）转移和MYCN状态，对结果的影响是否发生了变化。结果：随着时间的推移，结果有所改善：3年EFS 46%（1974-1989）和71%（1997-2002）。18个月对18个月的风险（HR 4.47）；骨髓转移（HR 4.00）；MYCN扩增（HR 3.97）。1997-2002年，BM受累和MYCN扩增的bb0 - 18个月的EFS为18%，但0-12个月无BM受累和MYCN扩增的EFS为89%。结论：有明确的证据表明，随着时间的推移，NB患儿的预后得到改善。提高诊断年龄的不利影响已经下降，但它仍然是不良预后的有力指标。这些结果支持将大于18个月的诊断年龄截止值作为INRG分类系统中的风险标准。(c) 2010 Elsevier Ltd.版权所有。
Purpose: Increasing age has been an adverse risk factor in children with neuroblastoma (NB) since the 1970's, with a 12-month age-at-diagnosis cut-off for treatment stratification. Over the last 30 years, treatment intensity for children > 12 months with advanced-stage disease has increased; to investigate if this strategy has improved outcome and/or reduced the prognostic influence of age, we analysed the International Neuroblastoma Risk Group (INRG) database.Patients and methods: Data from 11,037 children with NB (1974-2002) from Australia, Europe, Japan, North America. Cox modelling of event-free survival (EFS) tested if the era and prognostic significance of age-of-diagnosis, adjusted for bone marrow (BM) metastases and MYCN status, effects on outcome had changed.Results: Outcome improved over time: 3-year EFS 46% (1974-1989) and 71% (1997-2002). The risk for those > 18 months against 18 months (HR 4.47); BM metastases (HR 4.00); and MYCN amplified (HR 3.97). For 1997-2002, the EFS for > 18 months with BM involvement and MYCN amplification was 18%, but 89% for 0-12 months with neither BM involvement nor MYCN amplification.Conclusions: There is clear evidence for improving outcomes for children with NB over calendar time. The adverse influence of increasing age-at-diagnosis has declined but it remains a powerful indicator of unfavourable prognosis. These results support the age-of-diagnosis cut-off of greater than 18 months as a risk criterion in the INRG classification system. (c) 2010 Elsevier Ltd. All rights reserved.