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Neuroblastoma Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Treatment of High-Risk Neuroblastoma

The Children's Oncology Group (COG) high-risk group assignment criteria are described in Table 8.

Table 8. Children's Oncology Group (COG) Neuroblastoma High-Risk Group Assignment Schema Used for COG-P9641 and COG-A3961 Studiesa

INSS StageAgeMYCNStatusINPC ClassificationDNA Ploidyb
INPC = International Neuroblastoma Pathologic Classification; INSS = International Neuroblastoma Staging System.
a The COG-P9641 and COG-A3961 trials established the current standard of care for neuroblastoma patients in terms of risk group assignment and treatment strategies.
b DNA Ploidy: DNA Index (DI) > 1 is favorable, DI = 1 is unfavorable; hypodiploid tumors (with DI < 1) will be treated as a tumor with a DI > 1 (DI < 1 [hypodiploid] to be considered favorable ploidy).
c INSS stage 2A/2B symptomatic patients with spinal cord compression, neurologic deficits, or other symptoms are treated with immediate chemotherapy for four cycles.
d INSS stage 3 or stage 4 patients with clinical symptoms as listed above receive immediate chemotherapy.
e INSS 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.
2A/2Bc≥365 d–21 yAmplifiedUnfavorable-
3d<365 dAmplifiedAnyAny
≥365 d–21 yNonamplifiedUnfavorable-
≥365 d–21 yAmplifiedAny-
4d<365 dAmplifiedAnyAny
≥548 d–21 yAnyAny-
4Se<365 dAmplifiedAnyAny

Approximately 8% to 10% of infants with stage 4S disease will have MYCN-amplified tumors and are usually treated on high-risk protocols. The overall event-free survival (EFS) and overall survival (OS) for infants with stage 4 and 4S disease and MYCN-amplification were only 30% at 2 to 5 years posttreatment in a European study.[1]

For children with high-risk neuroblastoma, long-term survival with current treatments is about 54%.[2] Children with aggressively treated, high-risk neuroblastoma may develop late recurrences, some more than 5 years after completion of therapy.[3,4]

Standard Treatment Options for High-Risk Neuroblastoma

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