Table 2.
Clinical characteristics and relationship with local and distant metastases
| Mutated gene | SDHB | VHL | SDHC | SDHD | HIF2A | NF1 | RET |
|---|---|---|---|---|---|---|---|
| No. of patients | 25 | 21 | 1 | 1 | 2 | 1 | 2 |
| Median age (range), y | 13.3 (6.6-18) | 11 (2-18) | 16 | 11 | 10.1 (9.1-10.3) | 12 | 17 (14.6-18) |
| Sex: male | 13 | 14 | 0 | 0 | 1 | 1 | 2 |
| PHEO PGL | 7 18 | 17 4 | 0 1 | 0 1 | 1 1 | 0 1 | 2 0 |
| Metastasis at diagnosis | 1 | 2 | 1 | 0 | 1 | 1 | 0 |
| Event | 9 | 9 | 1 | 0 | 1 | 1 | 1 |
| PHEO PGL | 1 8 | 9 0 | 0 1 | 1 0 | 0 1 | 1 0 | |
| Median time to event, mo | 45 | 39 | 45 | 0 | 8 | 20 | 72 |
| Second non-PPGL tumor | 1 | ||||||
| Metachronous PPGL | – | 7 | 1 | – | – | – | |
| Local relapse | 7 | – | – | – | – | 1 | |
| Metastatic relapse | 2 | 1 | 0 | 1 | 1 | – |
Abbreviations: PHEO, pheochromocytoma; PGL, paraganglioma; PPGL, pheochromocytoma and paraganglioma.