Table 1.
Clinical characteristics of patients enrolled at the tertiary clinical centers
| Cluster 1 PPGL | Cluster 2 PPGL | Sporadic PPGL | |
|---|---|---|---|
| Number of patients | 80 | 94 | 224 |
| Age (years) at first diagnosis, median (IQR) | 28.7 (20.5-43.8) | 46.8 (31.9-59)a | 52.9 (42.5-62.4)a,b |
| Males | 47.5% (38/80) | 41.5% (39/94) | 44.6% (109/224) |
| Location | |||
| ȃAdrenal | 53.8% (43/80) | 98.9% (93/94)a | 94.2% (211/224)a |
| ȃExtra-adrenal | 46.3% (37/80) | 1.1% (1/94)a | 5.8% (13/224)a |
| Biochemical phenotype | |||
| ȃAdrenergic | 2.9% (2/68) | 91.4% (74/81)a | 62.8% (113/180)a,b |
| ȃNoradrenergic/dopaminergic | 97.1% (66/68) | 8.6% (7/81)a | 37.2% (67/180)a,b |
| Size of primary tumor (cm), medians (IQR) | 3.6 (2.6-4.8) | 3.5 (2.4-5.0) | 4.1 (2.8-5.8) |
| With recurrent disease | 47.5% (38/80) | 14.9% (14/94)a | 14.7% (33/224)a |
| ȃLocal recurrence/new tumor only | 22.5% (18/80) | 13.8% (13/94) | 6.7% (15/224)a |
| ȃWith metastasis | 25% (20/80) | 1.1% (1/94)a | 8% (18/224)a,b |
| Follow-up (years), median (IQR) | 8.7 (5.2-16.3) | 5.3 (3.1-9.8)a | 7 (4.6-10)a |
Abbreviations: IQR, interquartile range; PPGL, pheochromocytoma and paraganglioma.
Significantly different from cluster 1.
Significantly different from cluster 2.