Table 3.
Evaluation of the NBCCS criteria in patients with a medulloblastoma and germline SUFU mutation
| Sex, Age at MB, mo | Age at Last Follow-up, y | Falx Cerebri Calcification (age at CT scan) | Jaw Cysts or Dental Anomaly | BCC, Pits or Multiple Nevi | Macrocrania | Hypertelorism ± Frontal Bossing | Bifid Rib or Other Feature |
NBCCS
(age at diagnosis) |
|
|---|---|---|---|---|---|---|---|---|---|
| 1 | F, 17 | 28 | NA | No | Nevi (50) | Yes | No | NA, syndactyly of 2/3 toes | No |
| 2.A | M, 1 | 0 | No (1 mo) | NA | No | Yes | No | NA | No |
| 2.B | M, 3 | 0 | NA | NA | No | Yes | No | NA | No |
| 3 | M, 9 | 9 | No (1 y) | No | Nevi (20) | Yes | No | No | No |
| 4 | M, 15 | 11 | NA | High arched palate, dental agenesis | Palmar/plantar pits and nevi | Yes | No | No | Yes (8 y) |
| 5 | M, 30 | 34 | Yes (26 y) | No | BCC and nevi | Yes | No | No | Yes (17.5 y) |
| 6 | F, 31 | 4 | No (4 y) | High arched palate | No | Yes | Yes | No | No |
| 7 | M, 16 | 8 | No (2 y) | No | No | Yes | No | No | No |
| 8.A | M, 30 | 17 | No (4 y) | NA | No | Yes | No | NA | No |
| 8.B | M, 18 | 2 | NA | NA | No | Yes | No | NA | No |
| 8.C | M, 27 | 7 | No (7.5 y) | NA | No | Yes | No | NA | No |
| 9 | M, 11 | 1 | NA | No | No | Yes | No | NA | No |
| 10 | M, 8 | 9 | No (1 y) | NA | No | Yes | Yes | No, perinasal skin tag | No |
| 11 | F, 19 | 2 | Yes (2 y) | No | No | Yes | Yes | No | Yes (2 y) |
| 12.A | F, 9 | 17 | Yes (17 y) | No | No | Yes | No | No | Yes (17 y) |
| 12.B | F, 34 | 19 | NA | NA | Nevi | Yes | No | NA | No |
| 12.C | F, 19 | 4 | Yes, (3 y) | No | No | No | No | No | No |
| 13 | F, 6 | 3 | No (1 y) | No | No | Yes | No | No | No |
| 14 | F, 18 | 18 | Yes (16 y) | Dental agenesis | Nevi | Yes | No | No | Yes (16 y) |
| 15 | M, 1 | 2 | No | NA | No | Yes | No | No | No |
| 16 | F, 23 | 6 | No | No | No | No | No | No | No |
| 17 | M, 9 | 1 | No (1 y) | NA | No | Yes | No | No | No |