Clinicopathologic Characteristics
| Paraganglioma (n) | Genetic mutation (n) | |||||||||||||
| First author | Patients (n) | Mean age (y) | Subtype | Setting | Pheochromocytoma (n) | T&A | H&N | Multifocal/Metastatic (n) | Catecholamine-secretory (n) | SDHx | Others | Sporadic | Not tested | Tumor size (cm) |
| Archier (13) | 30 | 53 | No | S+R | 11 | 0 | 20 | 7 | NR | 8 | MAX: 1 | 21 | 0 | 2.0 |
| Chang (14) | 23 | 43 | No | S+R | 8 | 7 | 8 | 19 | 12 | 10 | 0 | 3 | 9 | NR |
| Janssen (15) | 20 | 48 | No | S | 0 | 0 | 20 | 17 | 12 | 16 | HIF2A: 1 | 3 | 0 | 2.2 |
| Kroiss (16) | 6 | 46 | No | S+R | 5 | 1 | 0 | 6 | NR | NR | NR | NR | 6 | NR |
| Kroiss (17) | 20 | 50 | No | S+R | 0 | 3 | 19 | 5 | NR | NR | NR | NR | 20 | NR |
| Naji (18) | 11 | NR | No | S | 7 | 2 | 2 | 2 | NR | 4 | 0 | 1 | 6 | 1.8 |
| Sharma (19) | 26 | 34 | No | S | 0 | 0 | 26 | 15 | 4 | NR | NR | NR | 26 | 3.4 |
| Sharma (20) | 62 | 34 | No | S | 62 | 0 | 0 | 7 | 54 | NR | RET*: 14 | NR | 62 | 4.1 |
| Tan (21) | 17 | 40† | No | S+R | 10 | NR | NR | 15 | 9 | NR | NR | NR | 17 | NR |
| Janssen (22) | 17 | 40 | SDHB mutation | R | 2 | 10 | 5 | 17 | 11 | 17 | 0 | 0 | 0 | NR |
| Janssen (23) | 22 | 50 | Sporadic | S+R | 13 | 9 | 0 | 22 | 19 | 0 | 0 | 22 | 0 | NR |
| Janssen (45) | 13 | 37 | PPGL–polycythemia | R | 7 | 7 | 0 | 4 | 13 | 0 | HIF2A: 6 | 6 | 0 | NR |
| PHD: 2 | ||||||||||||||
| Jha (24) | 9 | 17 | SDHx mutation | S+R | 1 | 6 | 2 | 9 | 7 | 9 | 0 | 0 | 0 | NR |
HIF2A = hypoxia-inducible factor 2A; H&N = head and neck; MAX = MYC-associated factor X; NR = not reported; PHD = prolyl hydroxylase; PPGL = pheochromocytoma and paraganglioma; R = restaging; S = staging; SDH = succinate dehydrogenase; T&A = thorax and abdomen.
↵* Multiple endocrine neoplasia type 2.
↵† Median.