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
|INSS Stage||Age||MYCNStatus||INPC Classification||DNA Ploidya|
|INPC = International Neuroblastoma Pathologic Classification; INSS = International Neuroblastoma Staging System.|
|a 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).|
|b INSS stage 2A/2B symptomatic patients with spinal cord compression, neurologic deficits, or other symptoms are treated with immediate chemotherapy for four cycles.|
|c INSS stage 3 or stage 4 patients with clinical symptoms as listed above receive immediate chemotherapy.|
|2A/2Bb||≥365 d–21 y||Amplified||Unfavorable||-|
|≥365 d–21 y||Nonamplified||Unfavorable||-|
|≥365 d–21 y||Amplified||Any||-|
|≥548 d–21 y||Any||Any||-|
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.
For children with high-risk neuroblastoma, long-term survival with current treatments is about 54%. Children with aggressively treated, high-risk neuroblastoma may develop late recurrences, some more than 5 years after completion of therapy.[3,4]
Treatment Options for High-Risk Neuroblastoma
Outcomes for patients with high-risk neuroblastoma remain poor despite recent improvements in survival in randomized trials.
A treatment option for high-risk neuroblastoma is the following:
- A regimen of chemotherapy, surgery, stem cell transplant (SCT), radiation therapy, and anti-GD2 antibody ch14.18 with interleukin-2/granulocyte-macrophage colony-stimulating factor (GM-CSF) and isotretinoin.
Chemotherapy, surgery, SCT, radiation therapy, and anti-GD2 antibody ch14.18, with interleukin-2/GM-CSF and isotretinoin
Treatment for patients with high-risk disease is generally divided into the following three phases:
- Induction (includes chemotherapy and surgical resection).
- Consolidation (hematopoietic stem cell rescue/transplantation [HSCT] and radiation therapy to the site of the primary tumor).
- Maintenance (immunotherapy and retinoid).