Table 2.
Demographics of Patients With a Clinical NF2 Diagnosis, Subdivided by Era of Diagnosis
| Year of Diagnosis of NF2 | P Value for Trend | ||||
|---|---|---|---|---|---|
| Era 1 <1998, n (%) | Era 21999-2008, n (%) | Era 3 2009-February 2020, n (%) | Total, n (%) | ||
| Number of patients | 113 | 115 | 125 | 353 | |
| Male | 55 (48.7) | 60 (52.2) | 57 (45.6) | 172 (48.7) | .618 |
| Female | 58 (51.3) | 55 (47.8) | 68 (54.4) | 181 (51.3) | |
| Median age diagnosis (y) | 24 | 20 | 27 | 23 | .175 |
| Bilateral VS | 104 (92.0) | 108 (93.9) | 94 (75.2) | 307 (86.7) | <.001 |
| Asymptomatic at diagnosis | 14 (12.3) | 24 (20.9) | 29 (23.2) | 67 (18.9) | .036 |
| No. with VS surgery/RTR in first 10 y | 72 (64.0) | 59 (51.3) | 39 (31.2) | 170 (48.3) | <.001 |
| Died | 39 (33.6) | 18 (15.7) | 3 (2.4) | 60 (16.7) | <.001 |
| Died within 10 y | 6 (5.3) | 10 (8.7) | 3 (2.4) | 19 (5.4) | .295 |
| Mean age at death (y) | 45.4 | 38.3 | 84.4 | 45.2 | .534 |
| Meningioma | 67 (59.7) | 52 (44.4) | 47 (36.8) | 166 (46.6) | .001 |
| Ependymoma | 31 (27.2) | 31 (27.0) | 25 (20.0) | 87 (24.6) | .178 |
| Bevacizumab | 13 (11.4) | 23 (21.7) | 22 (19.2) | 58 (17.5) | .219 |
| Meningioma surgery | 24 (20.2) | 17 (14.8) | 12 (11.2) | 53 (15.3) | .012 |
| Spinal surgery | 21 (18.4) | 18 (15.7) | 9 (7.2) | 48 (13.6) | .010 |
| Full constitutional Path variant | 82 (72.8) | 84 (73.0) | 60 (48.0) | 226 (64.1) | <.001 |
| Mosaic | 17 (14.9) | 21 (18.3) | 36 (28.8) | 74 (20.9) | .009 |
| De novo unknown probable mosaic | 13 (11.5) | 9 (7.8) | 27 (21.6) | 49 (13.9) | .021 |
| NF2 excluded (n) | 1 | 1 | 2 | 4 |
Abbreviations: n, number; %, percentage; VS, vestibular schwannoma; RTR, radiotherapy-related.