Table 1. Children’s Oncology Group (COG) Neuroblastoma Low-, Intermediate-, and High-Risk Group Assignment Schema Used for COG-9641 and COG-A3961 Studiesa
| INSS Stage | Age | MYCN Status | INPC Classification | DNA Ploidyb | Risk Group |
| 1 | 0–21 y | Any | Any | Any | Low |
| 2A/2Bc | <365 d | Any | Any | Any | Low |
| ≥365 d–21 y | Nonamplified | Any | - | Low | |
| ≥365 d–21 y | Amplified | Favorable | - | Low | |
| ≥365 d–21 y | Amplified | Unfavorable | - | High | |
| 3d | <365 d | Nonamplified | Any | Any | Intermediate |
| <365 d | Amplified | Any | Any | High | |
| ≥365 d–21 y | Nonamplified | Favorable | - | Intermediate | |
| ≥365 d–21 y | Nonamplified | Unfavorable | - | High | |
| ≥365 d–21 y | Amplified | Any | - | High | |
| 4d | <548 d [15-17] | Nonamplified | Any | Any | Intermediate |
| <365 d | Amplified | Any | Any | High | |
| ≥548 d–21 y | Any | Any | - | High | |
| 4Se | <365 d | Nonamplified | Favorable | >1 | Low |
| <365 d | Nonamplified | Any | =1 | Intermediate | |
| <365 d | Nonamplified | Unfavorable | Any | Intermediate | |
| <365 d | Amplified | Any | Any | High |
| INPC = International Neuroblastoma Pathologic Classification; INSS = International Neuroblastoma Staging System. | |||||
| aThe COG-9641 and COG-A3961 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; hypodiploid tumors (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 should be treated with immediate chemotherapy for four cycles. | |||||
| dINSS stage 3 or stage 4 patients with clinical symptoms as listed above should receive immediate chemotherapy. | |||||
| eINSS stage 4S infants with favorable biology and clinical symptoms should be treated with immediate chemotherapy until asymptomatic (2–4 cycles). Clinical symptoms include: 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. |
References
- Schmidt ML, Lal A, Seeger RC, et al.: Favorable prognosis for patients 12 to 18 months of age with stage 4 nonamplified MYCN neuroblastoma: a Children's Cancer Group Study. J Clin Oncol 23 (27): 6474-80, 2005. [PUBMED Abstract]
- George RE, London WB, Cohn SL, et al.: Hyperdiploidy plus nonamplified MYCN confers a favorable prognosis in children 12 to 18 months old with disseminated neuroblastoma: a Pediatric Oncology Group study. J Clin Oncol 23 (27): 6466-73, 2005. [PUBMED Abstract]
