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Table 2 Clinical characteristics of Neurofibromatosis type 2 patients in four studies

From: Neurofibromatosis type 2 (NF2): A clinical and molecular review

Characteristic Study    
  Kanter et al. [11] Evans et al. [1] Parry et al. [12] Mautner et al. [13]
Number of cases 73 120 63 48
Number of families 17 75 32 44
Sporadic cases 0 45 17 44
Mean age at onset (years) 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
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 NA 4.1 4.8 8
  1. 1In Mautner et al., the frequency of optic nerve sheath tumours is for all histological types (i.e., schwannomas and meningiomas).