Table 7. Children’s Oncology Group (COG) Neuroblastoma Low-Risk Group Assignment Schema Used for COG Studiesa

INSS Stage Age MYCN Status INPC Classification DNA Ploidyb
10–21 y AnyAnyAny
2A/2Bc<365 d Any AnyAny
≥365 d–21 y NonamplifiedAny-
≥365 d–21 yAmplifiedFavorable-
4Sd <365 dNonamplifiedFavorable>1

INPC = International Neuroblastoma Pathologic Classification; INSS = International Neuroblastoma Staging System.

aThe COG-P9641 (low risk) and COG-A3961 (intermediate risk) trials established the current standard of care for neuroblastoma patients in terms of risk group assignment and treatment strategies.

bDNA Ploidy: DNA Index (DI) > 1 is favorable, = 1 is unfavorable; a hypodiploid tumor (with DI < 1) will be treated as a tumor with a DI > 1 (DI < 1 [hypodiploid] to be considered favorable ploidy).

cINSS stage 2A/2B symptomatic patients with spinal cord compression, neurologic deficits, or other symptoms are treated with immediate chemotherapy for four cycles.

dINSS stage 4S infants with favorable biology and clinical symptoms are treated with immediate chemotherapy until asymptomatic (2–4 cycles). Clinical symptoms include the following: respiratory distress with or without hepatomegaly or cord compression and neurologic deficit or inferior vena cava compression and renal ischemia; or genitourinary obstruction; or gastrointestinal obstruction and vomiting; or coagulopathy with significant clinical hemorrhage unresponsive to replacement therapy.

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