Table 1.
Demographic and Tumor Characteristics of Pediatric and Adult Patients With PPGLs
| Characteristics | Pediatric | Adult | P Value |
|---|---|---|---|
| N | 95 | 653 | |
| Age at initial diagnosisa | 13.3 ± 3.5 | 44.7 ± 14.4 | |
| Male | 55.8% (53/95) | 48.1% (314/653) | 0.0980 |
| Primary tumor locations | |||
| Solitary adrenal | 22.1% (21/95) | 56.2% (367/653) | <0.0001 |
| Solitary extra-adrenal | 33.7% (32/95) | 21.6% (141/653) | <0.0001 |
| Bilateral adrenal | 11.6% (11/95) | 8.7% (57/653) | 0.2020 |
| Multifocalb | 32.6% (31/95) | 13.5% (88/653) | <0.0001 |
| Hereditary casesc | 80.4% (74/92) | 52.6% (273/519) | <0.0001 |
| Recurrent primary tumorsd | 29.5% (28/95) | 14.2% (93/653) | <0.0001 |
| Metastatic disease | 49.5% (47/95) | 29.1% (190/653) | <0.0001 |
| No. N/D phenotype | 93.2% (68/73) | 57.3% (337/588) | <0.0001 |
Abbreviations: N/D, noradrenergic/dopaminergic.
a
Age is shown as mean ± standard deviation.
b
Multifocal locations indicate multiple extra-adrenal tumors or extra-adrenal and adrenal tumors but exclude bilateral adrenal tumors unless accompanied by one or more extra-adrenal tumors.
c
Results were retrieved from 611 patients who underwent genetic testing.
d
Recurrent primary tumors are defined as recurrences at an original site of tumor resection as well as new primary tumors at other locations a year or more after diagnosis of the first primary tumor.