Table 1.
Clinical series of metastatic pheochromocytomas and paragangliomas
| Amar et al (Amar et al., 2007) | Ayala-Ramirez et al (Ayala-Ramirez et al., 2011) | Turkova et al (Turkova et al., 2016) | Hamidi et al (Hamidi et al., 2017) | Hescot et al (Hescot et al., 2019) | Fishbein et al (Fishbein et al., 2017) | |
|---|---|---|---|---|---|---|
| N | 54 | 131 | 132 | 272 | 169 | 71 |
| Timeframe | n/r | 1960–2009 | 2000–2014 | 1960–2016 | 1998–2010 | 200–2016 |
| PC/sPGL/HNPGL (%) | 54/46/0 | 52/48/0 | 29/71/0 | 40/47/11 | 53/37/10 | 28/45/211 |
| Synchronous metastasis | 24 (44%) | 67 (51%) | 26 (20%) | 96 (35%) | 79 (47%)2 | 18 (25%)3 |
| Age (Dx primary), y | 37.9 | n/r | 39 | n/r | 41 | |
| Age (Dx metastases), y | 42 | n/r | 44 | 48 | 6.2 (0–44.77)4 | |
| SDHB | 23/54 (43%) | 9/215 (43%) | 73/132 (55%) | 42/272 (15%)5 | 63/1515 (42%) | 37/605 (62%) |
| Died of disease | 26/54 (48%) | 87/131 (66%) | 39/132 (30%) | 73/272 (27%) | 72/169 (43%) | 19/71 (27%) |
| Age at death (range), y | 45 | n/r | 54 (7–91) | n/r | n/r |
1
an additional 5.6% had more than one tumor location;
2
metastases within 1 year;
3
metastases within 3 months;
4
years (range) after primary diagnosis;
5
not all cases had genetic testing; Dx primary: at first diagnosis, Dx metastases: at the time of metastases identification, n/r: not reported