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Algorithm for the COG Neuroblastoma Risk Classifier (version 2)

Algorithm for the COG Neuroblastoma Risk Classifier (version 2)
Risk classifier v2 algorithm for patients with (A) locoregional and (B) metastatic tumors. (A) Patients with locoregional tumors with neuroblastoma and ganglioneuroblastoma (nodular) are classified based on INRG stage (L1 and L2), age, resection, biomarkers (MYCN, ploidy, and SCAs), and INPC. Select patients with all favorable features are eligible for surveillance on the current non-high-risk COG ANBL1232 protocol. L1 or L2 tumors with histopathology diagnostic category of ganglioneuroma or ganglioneuroblastoma-intermixed will be classified as low risk regardless of biomarkers (and thus are not included in the figure). Ages are broken down by <18 months, 18 months to <5 years, and ≥5 years based on age categories used by INPC. (B) Patients with metastatic tumors are classified by stage (M and MS), age, INPC, and biomarkers. For MS patients, presence or absence of symptoms may influence therapy independent of biomarker status. In previous risk classifiers, missing data were considered as unfavorable. In COG v2, missing data for SCA will not be considered as unfavorable based on the low incidence of SCA in otherwise favorable subsets.

COG: Children's Oncology Group; GTR: gross-total resection; MYCN+: MYCN amplified; MYCN–: MYCN not amplified; FH: favorable histology; DI: DNA index; SCA: segmental chromosomal aberration; UH: unfavorable histology; INPC: International Neuroblastoma Pathology Classifier; v2: version 2; INRG: International Neuroblastoma Risk Group.

* If tumor progresses during observation, biopsy or resect and reclassify with biomarkers (as in COG ANBL1232).

¶ Consider complete resection if feasible.

Δ If no tumor burden symptoms, consider observation (as in COG ANBL1232).

◊ Biopsy contraindicated, defer biopsy until stable (NOTE: Biomarker results may modify risk class).

§ Hepatomegaly alone is an MS symptom in patients age <3 months of age (refer to COG ANBL1232).

¥ Response-based therapy (as in COG ANBL1232).

‡ MS score-based therapy (as in COG ANBL1232).
From: Irwin MS, Naranjo A, Zhang FF, et al. Revised Neuroblastoma Risk Classification System: A Report From the Children's Oncology Group. J Clin Oncol 2021; 39:3229. DOI: 10.1200/JCO.21.00278. Copyright © 2021 American Society of Clinical Oncology. Reproduced with permission from Wolters Kluwer Health. Unauthorized reproduction of this material is prohibited.
Graphic 138386 Version 1.0

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