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Neuroblastoma pp 271-292 | Cite as

Prognostic Factors and Risk Stratification

  • Meredith S. IrwinEmail author
Chapter

Abstract

Neuroblastoma (NB), the third most common paediatric cancer, accounts for 7% of paediatric malignancies and 10–15% of cancer-related deaths in children. Prognosis and tailored treatments are determined by several clinical and biological risk factors. These factors have been used to create risk classifiers that predict the probability of recurrence. The estimated 5-year survival rates for patients with non-high risk and high-risk neuroblastoma are 90% and 50%, respectively. Recent clinical trials have continued to reduce therapy for patients with non-high risk neuroblastoma, including the most favourable subsets of patients, who are now often followed with observation approaches. In contrast, high-risk neuroblastoma patients are treated aggressively with chemotherapy, radiation, surgery, myeloablative and immunotherapies. Current prognostic factors used by most clinical trials cooperative groups and the International Risk Group (INRG) classification system include age, stage, histopathology, ploidy, status of MYCN and segmental chromosome aberrations (SCAs). In addition to these well-validated risk factors, research advances facilitated by large international collaborations and next generation sequencing (NGS) technologies have identified additional emerging prognostic factors at diagnosis and during treatment. These include specific genetic alterations of the ALK oncogene, aberrations of the telomerase pathway (including TERT fusions and ATRX mutations) and metastatic response. Standard prognostic factors together with these newer biomarkers will enable us to further refine risk categories as well as optimize treatment and enable precision medicine strategies.

Keywords

Neuroblastoma Prognosis Risk stratification INRG MYCN Ploidy Segmental chromosome aberrations ALK Telomerase 

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© Springer Nature Switzerland AG 2020

Authors and Affiliations

  1. 1.Department of Paediatrics, Hospital for Sick ChildrenUniversity of TorontoTorontoCanada

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