Table 2.
Characteristics of the survivors and nonsurvivors among the 1226 NF1 patients whose vital status was known
| Clinical features* | Survivors n = 1159 No (%)† |
Nonsurvivors n = 67 No (%)† |
OR (95%CI)‡ |
P value* |
|---|---|---|---|---|
| Age, years, median [range] | 25.5 [2-74] | 31.7 [0-80] | - | 0.008 |
| Male gender | 548 (47) | 31 (46) | 1.0 (0.6-1.7) | 0.98 |
| Familial case (n = 897) | 498 (58) | 17 (53) | 0.7 (0.3-1.4) | 0.62 |
| At least 6 café-au-lait spots (1220) | 998 (86) | 48 (74) | 0.6 (0.3-1.1) | 0.07 |
| Freckles (n = 1217) | 985 (86) | 58 (88) | 1.1 (0.5-2.4) | 0.74 |
| Neurofibromas (n = 1215) | 863 (75) | 60 (90) | 3.1 (1.2-8.4 | 0.02 |
| Plexiform neurofibromas (n = 1209) | 333 (29) | 28 (42) | 1.8 (1.1-3.0) | 0.02 |
| Lisch nodules (n = 954) | 366 (40) | 16 (40) | 0.9 (0.5-1.9) | 0.89 |
| Renal artery stenosis (n = 601) | 5 (1) | 0 (0) | - | 0.60 |
| Scoliosis (n = 934) | 222 (25) | 9 (18) | 0.6 (0.3-1.3) | 0.23 |
| Nonunion (n = 1 194) | 43 (4) | 2 (3) | 0.7 (0.2-3.2) | 0.70 |
| Hydrocephalus (n = 1 190) | 31 (3) | 1 (2) | 0.6 (0.1-4.4) | 0.60 |
| OBNI (n = 526) | 202 (40) | 8 (30) | 0.6 (0.3-1.5) | 0.30 |
| Optic pathway glioma (n = 1 172) | 151 (13) | 11 (17) | 1.2 (0.6-2.6) | 0.58 |
| MPNST (n = 1198) | 44 (4) | 36 (55) | 31.2 (17.4-55.9) | <10-4 |
| Pheochromocytoma (n = 1193) | 10 (1) | 3 (5) | 4.3 (1.1-16.3) | 0.03 |
Abbreviations: OR, odds ratio; 95%CI, 95% confidence interval; OBNI, high T2-signal intensity lesions on magnetic resonance imaging of the brain; MPNST, malignant peripheral nerve sheath tumor
*Values in parentheses are the numbers of patients for whom data were available.
†Percentages may not total 100, because of missing data.
‡The ORS were adjusted for age.
Data are numbers (%) or median [range].