Neuroblastoma Treatment - National Cancer Institute

Table 1. Children’s Oncology Group Neuroblastoma Risk Group Assignment Schema

INSS Stage Age MYCN Status Shimada Classification DNA Ploidy^a Risk Group

1 0–21 y Any Any Any Low

2A/2B^b <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

3^c <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

4^c <548 d [13-15] Nonamplified Any Any Intermediate

<548 d Amplified Any Any High

≥548 d–21 y Any Any - High

4S^d <365 d Nonamplified Favorable >1 Low

<365 d Nonamplified Any =1 Intermediate

<365 d Nonamplified Unfavorable Any Intermediate

<365 d Amplified Any Any High

^aDNA 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).

^bINSS stage 2A/2B symptomatic patients with spinal cord compression, neurologic deficits, or other symptoms are treated on the LOW RISK NB Study with immediate chemotherapy for four cycles (course 1).

^cINSS stage 3 or stage 4 patients with clinical symptoms as listed above (or if in the investigator’s opinion it is in the best interest of the patient) will receive immediate chemotherapy.

^dINSS stage 4S infants with favorable biology and clinical symptoms are treated on the LOW RISK NB Study with immediate chemotherapy until asymptomatic (2–4 cycles). Clinical symptoms defined as: 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]