The challenge of defining "ultra-high-risk" neuroblastoma

Pediatr Blood Cancer. 2019 Apr;66(4):e27556. doi: 10.1002/pbc.27556. Epub 2018 Nov 26.

Abstract

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.

Keywords: biomarkers; high-risk; neuroblastoma; risk stratification.

Publication types

  • Review

MeSH terms

  • Disease-Free Survival
  • Humans
  • Neuroblastoma / diagnosis*
  • Neuroblastoma / mortality*
  • Neuroblastoma / therapy
  • Risk Assessment
  • Risk Factors
  • Survival Rate