Table 1.
Demographics: PHEOs/PGLs and controls
| PHEOs/PGLs | Controls | |
|---|---|---|
|
| ||
| No. | 71 | 142 |
|
| ||
| Age, years (median & range) | 40 (20-74) | 42 (20-74)† |
|
| ||
| Gender (M:F) | 32:39 | 67:75‡ |
|
| ||
| Type (%) | PGL: 40 (56%) | Healthy controls: 93 (65%) |
| PHEO: 26 (37%) | Benign diseases: 18 (13%) | |
| Mixed PHEO/PGL:5 (7%) | Other neoplasia: 31 (22%) | |
|
| ||
| Disease detectable | 70/71 (98.6%)ᶲ | N/A |
|
| ||
| Disease Extent | Local: 26 (37%) | N/A |
| Metastatic: 45 (63%) | ||
|
| ||
| Therapy | None: 18 (25%) | Chemotherapy: 6 (4%) |
| Surgery: 50 (70%) | Metformin 3 (2%) | |
| Embolization: 2 (3%) | PPI: 8 (6%) | |
| PRRT: 2 (3%) | ||
| Radiation: 16 (23%) | ||
| MIBG: 2 (3%) | ||
| Chemotherapy: 9 (13%) | ||
| Immunotherapy: 2 (3%) | ||
| SSA: 8 (11%) | ||
| Sunitinib: 1 (1%) | ||
| Temozolomide: 1 (1%) | ||
| TACE: 1 (1%) | ||
| Cryotherapy: 1 (1%) | ||
|
| ||
| Mutation status | EGLN1: 1 (1.4%) | N/A |
| FH: 1 (1.4%) | ||
| HIF2A: 1 (1.4%) | ||
| MAX: 1 (1.4%) | ||
| RET: 3 (4.2%) | ||
| RINT1: 1 (1.4%) | ||
| SH: 1 (1.4%) | ||
| SDHB: 25 (35.2%) | ||
| SDHC: 1 (1.4%) | ||
| SDHD: 3 (4.2%) | ||
| VHL: 1 (1.4%) | ||
| Negative: 19 (26.7%) | ||
| No data: 13 (18.3%) | ||
|
| ||
| Cluster assessment § | Cluster 1: 35 (49%) | N/A |
| Cluster 2: 4 (6%) | ||
| No cluster: 32 (45%) | ||
†
p=0.85 (Mann-Whitney U-test, 2 -tailed).
‡
p=0.88 (Fisher’s exact test, 2-tailed)
ᶲ
One patient exhibited biochemical evidence of recurrence
§
Per TCGA
Metaiodobenzylguanidine = MIBG; PHEO = pheochromocytoma; PGL = paraganglioma; PPI = Proton pump inhibitors; PRRT = peptide receptor radionuclide therapy; SSA = somatostatin analogs; TACE: Transcatheter arterial chemoembolization; TCGA = The Cancer Genome Atlas