Abstract
Neuroblastoma is a tumor with great clinical heterogeneity. Patients in North America are risk-stratified using a number of features including age at diagnosis, disease stage, tumor histology, MYCN status (amplified versus nonamplified), and tumor cell ploidy. In this paper, we review the evidence for utilizing these features in the risk classification of neuroblastic tumors. Additionally, we review the clinical and biologic criteria used by various cooperative groups to define low, intermediate, and high-risk disease populations in clinical trials, highlighting the differences in risk classification internationally. Finally, we discuss the development of the International Neuroblastoma Risk Group classification system, designed to begin worldwide standardization of neuroblastoma pretreatment risk classification and allow comparison of clinical trials conducted through different cooperative groups.
Highlights
One of the hallmarks of neuroblastoma is its clinical heterogeneity
Over the last several decades, numerous clinical and biologic factors have been incorporated into the risk classification system for neuroblastoma to categorize patients as having low, intermediate, or high-risk disease
Tumor ploidy was identified as a prognostic factor around the time the role of MYCN amplification in neuroblastoma was identified
Summary
One of the hallmarks of neuroblastoma is its clinical heterogeneity. While children with low-risk disease may be observed or undergo surgery and those with intermediate-risk disease may receive chemotherapy and undergo surgical resection, those with high-risk disease receive intensive, multimodality therapy that includes chemotherapy, surgery, myeloablative chemotherapy with autologous stem cell rescue, radiation, and immunotherapy with an anti-GD2 antibody [1,2,3]. (1) Age at diagnosis, (2) stage define extent of disease by International Neuroblastoma. Metastatic disease in children under 18 months of age is not associated with associated with the poor outcomes that result in older patients. Subgroup of patients with diploid, and for distant metastatic disease, the task force recommended an age MYCN cutoff ofnonamplified. 12 months (365 days)and for distant the INRG forINRG a subgroup of patients with diploid, tumors metastatic disease, analyses showed ageofretained prognostic significance in more modern thesystem. 12 months (365 days) for the INRG classification cohorts with intensified an older age cutoff of greater than 18 months system [4].treated. Cohorts treated with intensified therapy and supported an older age cutoff of greater than 18 months at diagnosis as a risk criterion [4,9]
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