期刊
EUROPEAN JOURNAL OF CANCER
卷 47, 期 4, 页码 561-571出版社
ELSEVIER SCI LTD
DOI: 10.1016/j.ejca.2010.10.022
关键词
Neuroblastoma; Outcome; Prognosis; Age-at-diagnosis; INRG
类别
资金
- William Guy Forbeck Research Foundation
- Little Heroes Pediatric Cancer Research Foundation
- Italian Neuroblastoma Foundation
- Italian Ministry of Health Ricerca Finalizzata - Bando Oncologia
- COG Group [NIH/NCI U10-CA98543]
- COG member institution [NIH/NCI U10-CA98413]
- COG Statistics and Data Center [NIH/NCI U10-CA29139, U10 CA98413]
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 <= 12 decreased: hazard ratio (HR); 4.61 and 3.94. For age 13-18 months, EFS increased from 42% to 77%. Outcome was worse if: > 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.
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