Hospital for Sick Children and University of Toronto, Toronto, Canada.
Children's Hospital of Philadelphia and University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania.
Pediatr Blood Cancer. 2019 Apr;66(4):e27556. doi: 10.1002/pbc.27556. Epub 2018 Nov 26.
Given the biological and clinical heterogeneity of neuroblastoma, risk stratification is vital to determining appropriate treatment. Historically, most patients with high-risk neuroblastoma (HR-NBL) have been treated uniformly without further stratification. Attempts have been made to identify factors that can be used to risk stratify these patients and to characterize an "ultra-high-risk" (UHR) subpopulation with particularly poor outcome. However, among published data, there is a lack of consensus in the definition of the UHR population and heterogeneity in the endpoints and statistical methods used. This review summarizes our current understanding of stratification of HR-NBL and discusses the complex issues in defining UHR neuroblastoma.
鉴于神经母细胞瘤的生物学和临床异质性，风险分层对于确定适当的治疗至关重要。历史上，大多数高危神经母细胞瘤（HR-NBL）患者未经进一步分层即进行统一治疗。人们试图确定可用于对这些患者进行风险分层的因素，并确定预后特别差的“超高危”（UHR）亚群。然而，在已发表的数据中，UHR 人群的定义缺乏共识，终点和所用统计方法存在异质性。本综述总结了我们目前对 HR-NBL 分层的理解，并讨论了定义 UHR 神经母细胞瘤的复杂问题。
