Clinical characteristics of NF2 patients in four studies
Characteristic | Kanter et al14 | Evans et al15 | Parry et al19 | Mautner et al20 |
---|---|---|---|---|
No of cases | 73 | 120 | 63 | 48 |
No of families | 17 | 75 | 32 | 44 |
Sporadic cases | 0 | 45 | 17 | 44 |
Mean age at onset (y) | 20 (of 59) | 22 | 20 | 17 |
Intracranial meningiomas (%) | 18 | 45 | 49 | 58 |
Spinal tumours (%) | NA | 26 | 67 | 90 |
Skin tumours (%) | 32 (of 73) | 68 (of 100) | 67 | 64 |
>10 skin tumours (%) | NA | 10 (of 100) | NA | NA |
Café au lait macules (%) | 42 (of 31) | 43 (of 100) | 47 | NA |
Cataract (%) | NA | 38 (of 90) | 81 | 62 |
Intracranial astrocytoma (%) | NA | 4.1 | 1.6 | NA |
Ependymoma (%) | NA | 2.5 | 3.2 | 6 |
Optic sheath meningioma (%)1-150 | NA | 4.1 | 4.8 | 8 |
↵1-150 In Mautner et al,20 the frequency of optic nerve sheath tumours is for all histological types (that is, schwannomas and meningiomas).