Table 1.
Clinical and biological features according to MYCN status in patients with neuroblastoma.
| Variable | All Patients N (%) | Heterogeneous MYCN Amplified N (%) | Homogeneous MYCN Amplified N (%) | p-value for Heterogeneous vs. Homogeneous MYCN | MYCN Wild-Type N (%) | p-value for Heterogeneous MYCN vs. Wild- type MYCN |
|---|---|---|---|---|---|---|
| Clinical Features | ||||||
| Male | 3,123 (52.29) | 29 (51.79) | 567 (57.80) | 0.3761 | 2,527 (51.20) | 0.9300 |
| Female | 2,850 (47.71) | 27 (48.21) | 414 (42.20) | 2,409 (48.80) | ||
| Unknown | 2 | 1 | -- | 1 | ||
| Age ≥18 months | 3,260 (54.56) | 35 (61.40) | 657 (66.97) | 0.3859 | 2,568 (52.02) | 0.1583 |
| Age <18 months | 2,715 (45.44) | 22 (38.60) | 324 (33.03) | 2,369 (47.98) | ||
| Unknown | -- | -- | -- | -- | ||
| INSS Stage 4 | 2,419 (40.64) | 33 (57.89) | 762 (77.76) | 0.0006 | 1,624 (33.04) | <0.0001 |
| All other stages | 3,533 (59.36) | 24 (42.11) | 218 (22.24) | 3,291 (66.96) | ||
| Unknown | 23 | -- | 1 | 22 | ||
| High-risk | 2,345 (40.22) | 48 (84.21) | 956 (98.35) | <0.0001* | 1,341 (27.93) | <0.0001 |
| Low/Intermediate-risk | 3,486 (59.78) | 9 (15.79) | 16 (1.65) | 3,461 (72.07) | ||
| Unknown | 144 | -- | 9 | 135 | ||
| Adrenal primary | 1,904 (33.53) | 21 (39.62) | 437 (47.04) | 0.2924 | 1,446 (30.79) | 0.1661 |
| Other primary sites | 3,775 (66.47) | 32 (60.38) | 492 (52.96) | 3,251 (69.21) | ||
| Unknown | 296 | 4 | 52 | 240 | ||
| Thoracic primary | 1,080 (19.02) | 9 (16.98) | 18 (1.94) | <0.0001* | 1,053 (22.42) | 0.3448 |
| Other primary sites | 4,599 (80.98) | 44 (83.02) | 911 (98.06) | 3,644 (77.58) | ||
| Unknown | 296 | 4 | 52 | 240 | ||
| High LDH (≥662.5 U/mL) | 992 (48.65) | 16 (76.19) | 356 (91.05) | 0.0424* | 620 (38.11) | 0.0004 |
| Normal LDH (<662.5 U/mL) | 1,047 (51.35) | 5 (23.81) | 35 (8.95) | 1,007 (61.89) | ||
| Unknown | 3,936 | 36 | 590 | 3,310 | ||
| High ferritin (>115 ng/mL) | 619 (47.84) | 10 (62.50) | 175 (75.11) | 0.2528* | 434 (41.53) | 0.0915 |
| Normal ferritin (<115 ng/mL) | 675 (52.16) | 6 (37.50) | 58 (24.89) | 611 (58.47) | ||
| Unknown | 4,681 | 41 | 748 | 3,892 | ||
| Biologic Features | ||||||
| Diploid | 1,894(36.93) | 22 (45.83) | 503 (61.49) | 0.0309 | 1,369(32.12) | 0.0433 |
| Hyperdiploid | 3,234 (63.07) | 26(54.17) | 315(38.51) | 2,893 (67.88) | ||
| Unknown | 847 | 9 | 163 | 675 | ||
| LOH at 1p | 494(21.41) | 5 (26.32) | 267 (74.37) | <0.0001 | 222(11.51) | 0.0608* |
| No 1pLOH | 1,813(78.59) | 14(73.68) | 92 (25.63) | 1,707 (88.49) | ||
| Unknown | 3,668 | 38 | 622 | 3,008 | ||
| LOH at 11q | 350(15.19) | 2(10.53) | 25 (6.94) | 0.6361* | 323(16.78) | 0.7564* |
| No 11qLOH | 1,954(84.81) | 17(89.47) | 335 (93.06) | 1,602 (83.22) | ||
| Unknown | 3,671 | 38 | 621 | 3,012 | ||
| LOH at 1p and/or 11q | 791 (33.86) | 6(31.58) | 271 (75.49) | <0.0001 | 514(26.25) | 0.6037* |
| No LOH at 1p or 11q | 1,545(66.14) | 13(68.42) | 88(24.51) | 1,444 (73.75) | ||
| Unknown | 3,639 | 38 | 622 | 2,979 | ||
| ALK aberration | 65 (32.34) | --- | 42 (47.73) | 0.0616* | 23(21.30) | 0.5813* |
| No ALK aberration | 136(67.66) | 5(100.0) | 46 (52.27) | 85 (78.70) | ||
| Unknown | 5,774 | 52 | 893 | 4,829 | ||
| Unfavorable histology | 2,604 (46.30) | 32 (62.75) | 856 (92.94) | <0.0001* | 1,716(36.89) | 0.0001 |
| Favorable histology | 3,020 (53.70) | 19(37.25) | 65 (7.06) | 2,936(63.11) | ||
| Unknown | 351 | 6 | 60 | 285 | ||
| High MKI | 943(18.91) | 18(42.86) | 589(68.81) | 0.0005 | 336 (8.22) | <0.0001* |
| Low/Intermediate MKI | 4,043(81.09) | 24(57.14) | 267(31.19) | 3,752 (91.78) | ||
| Unknown | 989 | 15 | 125 | 849 | ||
| Undifferentiated/Poorly Differentiated | 4,753(91.77) | 45 (93.75) | 940(99.16) | 0.0132* | 3,768 (90.08) | 0.6235* |
| Differentiating | 426 (8.23) | 3 (6.25) | 8 (0.84) | 415(9.92) | ||
| Unknown | 796 | 9 | 33 | 754 | ||
Abbreviations: INSS=International Neuroblastoma Staging System; LDH=lactate dehydrogenase; LOH=loss of heterozygosity; MKI=mitosis karyorrhexis
*
Fisher’s exact test was used in place of chi-squared test