Table 1.
Clinical and Genetic Characteristics and Postrelapse Survival in the Relapsed INRG Cohort (n = 2,266)
| Factor | Patients |
Postrelapse OS |
5-Year Postrelapse OS |
Postrelapse OS P† | ||
|---|---|---|---|---|---|---|
| No. | % | HR* | 95% CI | OS ± SE (%) | ||
| Histologic classification‡ | 4.5 | 3.6 to 5.8 | < .001 | |||
| Favorable | 246 | 27 | 66 ± 5 | |||
| Unfavorable | 653 | 73 | 11 ± 2 | |||
| INSS stage | 3.5 | 3.1 to 4.0 | < .001 | |||
| 1, 2, 3, 4S | 622 | 28 | 52 ± 3 | |||
| 4 | 1,578 | 72 | 8 ± 1 | |||
| MYCNstatus | 2.7 | 2.4 to 3.1 | < .001 | |||
| Nonamplified | 1,141 | 67 | 32 ± 2 | |||
| Amplified | 562 | 33 | 7 ± 2 | |||
| Initial treatment | 2.6 | 2.3 to 2.9 | < .001 | |||
| Observation, surgery, or standard chemotherapy | 762 | 40 | 41 ± 3 | |||
| Intensive multimodality | 1,143 | 60 | 7 ± 1 | |||
| Age, days | 2.4 | 2.1 to 2.7 | < .001 | |||
| < 547 | 617 | 27 | 47 ± 3 | |||
| ≥ 547 | 1,649 | 73 | 10 ± 1 | |||
| LDH, U/L | 2.4 | 2.1 to 2.8 | < .001 | |||
| < 580 | 466 | 35 | 38 ± 2 | |||
| ≥ 580 | 848 | 65 | 12 ± 2 | |||
| Serum ferritin, ng/mL | 2.1 | 1.8 to 2.4 | < .001 | |||
| < 96 | 321 | 28 | 34 ± 4 | |||
| ≥ 96 | 839 | 72 | 12 ± 2 | |||
| MKI‡ | 1.9 | 1.6 to 2.4 | < .001 | |||
| Low, intermediate | 550 | 74 | 28 ± 3 | |||
| High | 189 | 26 | 12 ± 4 | |||
| 1p | 1.8 | 1.4 to 2.1 | < .001 | |||
| No loss or aberration | 345 | 58 | 32 ± 5 | |||
| LOH, deletion, or imbalance | 252 | 42 | 15 ± 3 | |||
| Ploidy | 1.6 | 1.4 to 2.0 | < .001 | |||
| > 1 (hyperdiploid) | 357 | 57 | 34 ± 4 | |||
| ≤ 1 (diploid, hypodiploid) | 273 | 43 | 16 ± 4 | |||
| Grade of NB differentiation‡ | 1.6 | 1.1 to 2.1 | < .001 | |||
| Differentiating | 72 | 9 | 38 ± 8 | |||
| Undifferentiated | 774 | 91 | 22 ± 3 | |||
| Diagnostic category‡ | ||||||
| 1 (NB, stroma poor) | 992 | 91 | 21 ± 2 | |||
| 2 (GNB, intermixed, stroma rich) | 5 | 0.5 | 0 | |||
| 3 (GNB, well differentiated, stroma rich) | 5 | 0.5 | 0 | |||
| 4 (GNB, nodular [composite]) | 90 | 8 | 10 ± 7 | |||
| 2 and 3 v 1 and 4 | 1.4 | 0.8 to 2.4 | .686 | |||
| 2 and 3 | 10 | 1 | 0 | |||
| 1 and 4 | 1,082 | 99 | 20 ± 2 | |||
| Year of diagnosis | 1.3 | 1.2 to 1.4 | < .001 | |||
| ≥ 1996 | 1,333 | 59 | 25 ± 3 | |||
| < 1996 | 933 | 41 | 17 ± 1 | |||
| 11q | 1.1 | 0.8 to 1.5 | .567 | |||
| Balanced or no aberration | 205 | 65 | 35 ± 6 | |||
| Deletion, imbalance, or unbalanced | 115 | 35 | 20 ± 7 | |||
| 17q | 1.1 | 0.7 to 1.6 | .764 | |||
| No gain | 64 | 41 | 29 ± 8 | |||
| Gain | 91 | 59 | 23 ± 9 | |||
Abbreviations: GNB, ganglioneuroblastoma; HR, hazard ratio; INPC, International Neuroblastoma Pathology Classification; INRG, International Neuroblastoma Risk Group; INSS, International Neuroblastoma Staging System; LDH, lactate dehydrogenase; LOH, loss of heterozygosity; MKI, mitosis karyorrhexis index; NB, neuroblastoma; OS, overall survival.
*
HRs denote increased risk of event for second row within given category as compared with first row.
†
Per log-rank test.
‡
Per INPC; per Shimada, if INPC missing.