Table 1.
Ages of diagnosis and locations of tumors in patients with and without established mutations or hereditary syndromes
| MEN 2 | Established mutation or hereditary syndrome |
SDHD | No established mutation or hereditary syndrome |
||||
|---|---|---|---|---|---|---|---|
| NF 1 | VHL | SDHB | EPI | No EPI | |||
| No. | 38 | 10 | 66 | 48 | 11 | 95 | 87 |
| Age data | a | ab | ab | a | a | ||
| Mean ± 95% CI | 39.6 ± 4.1 | 42.1 ± 7.9 | 30.6 ± 4.1 | 31.3 ± 4.2 | 30.7 ± 8.8 | 50.4 ± 3.0c | 42.4 ± 3.2 |
| Median | 36.1 | 43.2 | 28.5 | 32.4 | 23.3 | 50.3 | 41.6 |
| Range | (16–75) | (17–59) | (6–75) | (7–70) | (12–59) | (18–78) | (11–83) |
| 10th & 90th percentiles | (27–54) | (26–55) | (10–55) | (11–50) | (20–53) | (31–71) | (24–64) |
| Tumor location (A/E/B) | 38/0/0 | 9/1/0 | 58/5/3 | 2/43/3 | 1/5/5 | 91/4/0 | 54/30/3 |
Patients without an established mutation were divided into two groups according to whether their tumors produced appeciable epinephrine (EPI) or did not produce appreciable epinephrine (no EPI) as outlined in Fig. 1.
Tumor locations indicate adrenal (A), extradrenal (E), or both adrenal and extraadrenal (B) primary tumors.
Denotes a lower age (P < 0.05) than EPI.
Denotes a lower age (P < 0.05) than No EPI.