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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).