Table 2.
Clinical characteristics of a subgroup of patients enrolled at the tertiary clinical centers, as defined by the following 2 criteria: absence of previous history of PPGL and a follow-up duration of more than 5 years
| Cluster 1 PPGL | Cluster 2 PPGL | Sporadic PPGL | |
|---|---|---|---|
| Number of patients | 26 | 27 | 96 |
| Age (years) at first diagnosis, median (IQR) | 27.2 (16.5-45.9) | 48.8 (28.2-58.9)a | 53.4 (44.3-62.7)a |
| Males | 42.3% (11/26) | 33.3% (9/27) | 39.6% (38/96) |
| Location | |||
| ȃAdrenal | 65.4% (17/26) | 96.3% (26/27)a | 95.8% (92/96)a |
| ȃExtra-adrenal | 34.6% (9/26) | 3.7% (1/27)a | 4.2% (4/96)a |
| Biochemical phenotype | |||
| ȃAdrenergic | 0 | 88.9% (24/27)a | 65.6% (63/96)a |
| ȃNoradrenergic/dopaminergic | 100% (26/26) | 11.1% (3/27)a | 34.4% (33/96)a |
| Size of primary tumor (cm), median (IQR) | 3.7 (2.5-4.3) | 3.6 (2-4.5) | 4 (2.9-5.7) |
| With recurrent disease | 23.1% (6/26) | 11.1% (3/27) | 6.3% (6/96)a |
| ȃLocal recurrence/new tumor only | 11.5% (3/26) | 11.1% (3/27) | 5.2% (5/96) |
| ȃWith metastasis | 11.5% (3/26) | 0 | 1.0% (1/96)a |
| Follow-up (years), median (IQR) | 8.2 (6.2-10.4) | 8.2 (5.6-11.4) | 7.3 (6.2-9.5) |
Abbreviations: IQR, interquartile range; PPGL, pheochromocytoma and paraganglioma.
Significantly different from cluster 1.