Table 1.

Risk Groups according to Revised COG Risk Classifier, 20211: Shown are estimated survival ranges for risk groups, patient cohorts (by age, stage, and tumor biology) classified as low, intermediate and high risk, and general treatment approaches.

Risk Group	Low-Risk	Intermediate-Risk	High-Risk
Survival	OS: ≥95%	OS: ≥85%	EFS: 50 - <80%
Patient/Tumor Characteristics	• L1 (except MYCN-A incompletely resected) • MS <12 mo MYCN-NA, INPC-fav, No SCA, no symptoms	• L2 <18mo MYCN-NA • L2 >18 mo MYCN-NA, INPC-fav • M <12mo MYCN-NA • M or MS, 12–18 mo MYCN-NA, INPC-fav, No SCA • MS <12mo MYCN-NA, INPC-unfav OR SCA+ OR diploid	• L2, M, MS - MYCN-A (any age) • M or MS, 12–18 mo- MYCN-NA AND INPC-unfav OR SCA+ OR diploid • M >18mo (any biology)
Typical Treatment Approaches	Observation OR Surgery Chemotherapy only for MS-related symptoms, cord compression	2–8 cycles lower intensity chemotherapy (based on stage, tumor biology) +/− surgery	Induction: Chemo, Surgery Consolidation: ASCT(s)*, radiation therapy Post Consolidation: Anti-GD2 immunotherapy

MYCN-NA, MYCN non-amplified: MYCN-A, MYCN amplified: INPC, International Neuroblastoma Pathology Classification; fav, favorable; unfav, unfavorable; SCAs, segmental chromosome aberrations

^*Note: not all high-risk patients are eligible for tandem transplant on recent protocols