Table 1.
Demographics and disease characteristics of study participants.
| Characteristics | Value Total | % SDHx | % Sporadic |
|---|---|---|---|
| No. of participants | 36 (100) | 18 (100) | 18 (100) |
| No. of females | 16 (44.4) | 7 (38.9) | 9 (50.0) |
| No. of males | 20 (55.6) | 11 (61.1) | 9 (50.0) |
| Race | |||
| Black/African American | 6 (16.7) | 1 (5.5) | 5 (27.8) |
| White | 28 (77.8) | 16 (88.9) | 12 (66.7) |
| Hispanic or multiracial | 2 (5.5) | 1 (5.5) | 1 (5.5) |
| Age at diagnosis (y) | 43.6 ± 14.7 | 39.2 ± 12.7 | 48.0 ± 15.6 |
| Age at metastasis (y) | 49.3 ± 15.0 | 44.3 ± 13.1 | 54.3 ± 15.5 |
| Age at enrollment (y) | 53.2 ± 14.3 | 49.1 ± 13.1 | 57.2 ± 14.6 |
| Time between diagnosis and development of metastases (y) | 5.6 ± 7.2 | 5.1±6.1 | 6.3±8.4 |
| No. of participants who were metastatic at the time of diagnosis | 10 (27.7) | 4 (22.3) | 6 (33.3) |
| Size of the primary tumor (cm) | 8.3 ± 4.8 | 8.3 ± 4.3 | 8.3 ± 5.5 |
| Location of the primary tumor | |||
| Pheochromocytoma only | 9 (25.0) | 0 (0) | 9 (50.0) |
| Paraganglioma (head and neck) only | 3 (8.3) | 1 (5.5) | 2 (11.1) |
| Paraganglioma (extraadrenal) only | 19 (52.8) | 14 (77.8) | 5 (27.8) |
| Both head and neck as well as extraadrenal paraganglioma | 1 (2.8) | 1 (5.5) | 0 (0) |
| Both pheochromocytoma and paraganglioma (all) | 4 (11.1) | 2 (11.1) | 2 (11.1) |
| Biochemical elevations | |||
| Epinephrine/metanephrine | 9 (25) | 4 (22.2) | 5 (27.8) |
| Norepinephrine/normetanephrine | 27 (75) | 12 (66.7) | 15 (83.3) |
| Dopamine | 15 (41.7) | 8 (44.4) | 7 (38.9) |
| Chromogranin A | 33 (91.7) | 17 (94.4) | 16 (88.9) |
| Location of the metastasis | |||
| Lung | 16 (44.4) | 6 (33.3) | 10 (55.5) |
| Liver | 21 (58.3) | 9 (50.0) | 12 (66.7) |
| Bone | 31 (86.1) | 16 (88.9) | 15 (83.3) |
| Lymph nodes | 18 (50.0) | 10 (55.5) | 8 (44.4) |
| Lung or liver or peritoneal/omental/mesenteric deposits | 27 (75.0) | 13 (72.2) | 14 (77.8) |
| Treatment received | |||
| Surgery | 30 (83.3) | 16 (88.9) | 14 (77.8) |
| Chemotherapy and/or TKIs | 11 (30.5) | 6 (33.3) | 5 (27.8) |
| Cold somatostatin analogs (SSAs) | 10 (27.8) | 5 (27.8) | 5 (27.8) |
| External radiation therapy | 17 (47.2) | 12 (66.7) | 5 (27.8) |
| Liver-directed therapies | 6 (16.7) | 6 (33.3) | 0 (0) |
| Bone-directed therapies (bisphosphonates or denosumab) | 6 (16.7) | 5 (27.8) | 1 (5.5) |
| No. of prior systemic treatments | |||
| 0 | 19 (52.8) | 9 (50.0) | 10 (55.5) |
| 1 | 12 (33.3) | 6 (33.3) | 6 (33.3) |
| 2 or more | 5 (13.9) | 3 (16.7) | 2 (11.1) |
TKI = tyrosine kinase inhibitors, SSA = somatostatin analogs.