Neuroblastoma Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Treatment of Intermediate-Risk Neuroblastoma
The Children's Oncology Group (COG) intermediate-risk group assignment criteria are described in Table 7.
Table 7. Children's Oncology Group (COG) Neuroblastoma Intermediate-Risk Group Assignment Schema Used for COG-P9641 and COG-A3961 Studiesa
|INSS Stage||Age||MYCNStatus||INPC Classification||DNA 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 3 or stage 4 patients with clinical symptoms as listed above receive immediate chemotherapy.|
|d 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.|
|≥365 d–21 y||Nonamplified||Favorable||-|
(Refer to the Treatment of Stage 4S Neuroblastoma section of this summary for more information about the treatment of stage 4S neuroblastoma.)
Standard Treatment Options for Intermediate-Risk Neuroblastoma
Standard treatment options for intermediate-risk neuroblastoma include the following:
- Surgery and chemotherapy.
- Surgery and observation (in infants).
- Radiation therapy.
Surgery and chemotherapy
Patients categorized as intermediate risk have been successfully treated with surgery and four to eight cycles of chemotherapy (carboplatin, cyclophosphamide, doxorubicin, and etoposide; the cumulative dose of each agent is kept low to minimize permanent injury from the chemotherapy regimen) (COG-A3961). As a rule, patients whose tumors had unfavorable biology received eight cycles of chemotherapy, compared with four cycles for patients whose tumors had favorable biology. The COG-A3961 phase III trial demonstrated that therapy could be significantly reduced for patients with intermediate-risk neuroblastoma while maintaining outstanding survival.