Total | Hereditary proven disease | Sporadic disease | Disease associated with not clearly deleterious mutations of SDHs | p value | Phaeo or Pgl associated with VHL | Phaeo or Pgl associated with RET | Tumours associated with SDHs | |
---|---|---|---|---|---|---|---|---|
The p values are for the comparison between hereditary disease and sporadic disease. | ||||||||
Number of patients | n=36 | n=17 | n=15 | n=4 | n=13 | n=1 | n=3 | |
Age at presentation (y): | ||||||||
Mean | 33.6 | 29.1 | 36.7 | 41.2 | NS | 30.0 | 23.0 | 27.3 |
SD | 13.7 | 11.5 | 14.7 | 15.6 | 13.0 | – | 4.6 | |
Range | 13–63 | 13–49 | 14–63 | 19–53 | 13–47 | – | 22–30 | |
≤13 | 2 | 2 (11.8%) | 0 | 0 | 2 (15.4%) | 0 | ||
≤18 | 7 | 4 (23.5%) | 3 (20%) | 0 | NS | 4 (30.8%) | 0 | |
≥50 | 6 | 0 (0%) | 4 (26.7%) | 2 (50%) | NS | 0 | 0 | |
Type of tumour: | ||||||||
Single | 23 | 7 (30.4%) | 12 (52.2%) | 4 (17.4%) | <0.05 (χ2) | 5 (38.5%) | 2 (66.6%) | |
Multifocal | 13 | 10 (76.9%) | 3 (23.1%) | 0 | <0.05 (χ2) | 8 (61.5%) | 1 (100%) | 1 (33.3%) |