Table 2:
Clinical Characteristics of the reviewed patients
| Patients (n=703) | Frequency | % | |
|---|---|---|---|
| Gender | Male | 307 | 43.7 |
| Female | 392 | 55.7 | |
| Data not available | 4 | 0.6 | |
| Age at diagnosis | Median (range) | 46 (7–84) | |
| Tumor size mm, | Median (range) | 45 (10–160) | |
| Stage | Non-metastatic | 618 | 87.9 |
| Metastatic | 85 | 12.1 | |
| Catecholamine profile | Epinephrine | 161 | 22.9 |
| Norepinephrine | 139 | 19.8 | |
| Dopamine | 24 | 3.4 | |
| Data not available | 324 | 53.9 | |
| WHO 2004 | Pheochromocytoma | 572 | 81.4 |
| Paraganglioma | 127 | 18.1 | |
| Data not available | 4 | 0.5 | |
| WHO 2017 | PCC | 572 | 81.4 |
| Sympathetic PGL | 96 | 13.7 | |
| Head and Neck PGL | 27 | 3.8 | |
| Data not available | 8 | 1.1 | |
| Time on follow up (months) | Median (range) | 33 (0–316) | |
| Status at the end of follow-up | Alive | 494 | 70.3 |
| Dead | 40 | 5.7 | |
| Data not available | 169 | 24.0 | |
| ATRX mutation status | ATRX mutated | 450 | 63.9 |
| ATRX wild type | 17 | 2.4 | |
| Data not available | 237 | 33.7 | |
| 2-molecular subgroups | SDHB wild type | 645 | 91.7 |
| SDHB mutated | 58 | 8.3 | |
| 3-molecular subgroups | Pseudohypoxia | 177 | 25.2 |
| Kinase signaling | 245 | 34.9 | |
| Wnt/unknown | 281 | 39.9 | |
| 4-molecular subgroups | Pseudohypoxia TCA-cycle | 79 | 11.3 |
| Pseudohypoxia VHL/EPAS1 | 98 | 13.9 | |
| Kinase signaling | 245 | 34.9 | |
| Wnt/unknown | 281 | 39.9 | |
DA, Dopamine; E, Epinephrine; F, Female; HNPGL, Head and Neck PGL; M, Male; NA, Not Available; NE, Norepinephrine; PCC, Pheochromocytoma; PGL, Paraganglioma; sPGLs Sympathetic PGL; TCA, tricarboxylic acid.
Data on age was missing in 3 patients, on tumor size on 291 patients and on follow up length in 167 patients.