Table 1.
Patient Demographic and Clinical Characteristics
| All Schwannomatosis Patients (n=97) | Confirmed Schwannomatosis Patients (n=64) | Probable Schwannomatosis Patients (n=33) | |
|---|---|---|---|
| Female | 49 (50.5%) | 30 (46.9%) | 19 (57.6%) |
| Familial Disease | 15 (15.4%) | 11 (17.2%) | 4 (12%) |
| Anatomically Limited Disease | 20 (20.6%) | 14 (21.9%) | 6 (18%) |
| Age at First Symptom, median (range) | 32.9 years (5.1 – 68.3 years) | 30.8 years (5.1 – 64.1 years) | 39.7 years (12.5 – 68.3 years) |
| First Symptom Type | |||
| Pain | 51 (52.6%) | 34 (53.1%) | 17 (51.5%) |
| Palpable Mass | 10 (10.3%) | 7 (10.9%) | 3 (9.1%) |
| Neurological Symptoms† | 4 (4.1%) | 0 (0.0%) | 4 (12.1%) |
| Pain and Palpable Mass | 11 (11.3%) | 6 (9.4%) | 5 (15.2%) |
| Pain and Neurological Symptoms | 8 (8.2%) | 7 (10.9%) | 1 (3.0%) |
| Other‡ | 1 (1.0%) | 1 (1.6%) | 0 (0.0%) |
| Unknown | 2 (2.1%) | 2 (3.1%) | 0 (0.0%) |
| N/A: Asymptomatic | 10 (10.3%) | 7 (10.9%) | 3 (9.1%) |
| First Symptom Frequency | |||
| Constant | 33 (34.0%) | 21 (32.8%) | 12 (36.4%) |
| Intermittent | 29 (29.9%) | 18 (28.1%) | 11 (33.3%) |
| Unknown | 25(25.8%) | 18 (28.1%) | 7 (21.2%) |
| N/A: Asymptomatic | 10 (10.3%) | 7 (10.9%) | 3 (9.1%) |
| Initial Imaging Workup | |||
| Single tumor discovered | 54 (55.7%) | 38 (59.4%) | 16 (48.5%) |
| Two or more tumors discovered | 40 (41.2%) | 25 (39.1%) | 15 (45.4%) |
| Unknown | 2 (2.1%) | 1 (1.6%) | 1 (3.0%) |
| N/A – no tumors discovered | 1 (1.0%) | 0 (0%) | 1 (3.0%) |
Footnotes:
†
Neurological symptoms included numbness, paresthesias, muscle weakness and hypophonia.
‡
Other symptom was severe coughing spells due to a schwannoma compressing the esophagus.