Table 3.
Demographics of Patients With a Clinical NF2 Diagnosis, Subdivided by Mode of Inheritance
| Inheritance Type | P Value | ||||
|---|---|---|---|---|---|
| De Novo Constitutional, n (%) | De Novo Mosaic, n (%) | De Novo Unknown, n (%) | Inherited, n (%) | ||
| Number | 106 | 74 | 49 | 120 | n/a |
| Female | 46 (43.4) | 47 (63.5) | 29 (59.2) | 59 (49.2) | .037 |
| Bilateral VS | 104 (98.1) | 55 (74.3) | 38 (77.6) | 109 (90.9) | <.001 |
| Median age diagnosis (y) | 19 | 35 | 48 | 16 | <.001 |
| VS surgery/RTR in first 10 y | 71 (67.0) | 39 (52.7) | 15 (30.6) | 42 (34.7) | <.001 |
| Died | 28 (26.4) | 7 (9.5) | 6 (12.2) | 19 (15.8) | .015 |
| Died within 10 y | 11 (10.4) | 2 (2.7) | 1 (2.0) | 5 (4.1) | .056 |
| Mean age at death (y) | 37.2 | 54.4 | 63.3 | 47.9 | .003 |
| Meningioma | 61 (57.5) | 39 (52.7) | 20 (40.8) | 46 (38.0) | .018 |
| Ependymoma | 43 (40.6) | 13 (17.6) | 3 (6.1) | 28 (23.3) | <.001 |
| Bevacizumab | 25 (23.5) | 5 (6.8) | 1 (2.0) | 27 (22.5) | <.001 |
| Meningioma surgery | 24 (22.6) | 10 (13.5) | 4 (8.2) | 15 (12.5) | .063 |
| Spinal surgery | 21 (19.8) | 11 (14.9) | 3 (6.1) | 13 (10.7) | .085 |
Abbreviations: n, number; %, percentage; VS, vestibular schwannoma; RTR, radiotherapy-related.
Four disputed diagnoses have been removed.