Table 1 Demographic and clinical characteristics of the ECD patients according to their BRAF status
Characteristic | Total cohort (n = 32) | BRAFV600E mutation (n = 21) | BRAF WT (n = 9) | P value |
|---|---|---|---|---|
Age at diagnosis, years (median, range) | 48 (6–66) | 52 (6–66) | 37 (32–56) | NS |
Number of involved organs (median, range) | 4 (1–8) | 5 (2–8) | 2 (1–7) | NS |
organs of involvement | ||||
Bone | 30 (93.8%) | 20 (95.2%) | 8 (88.9%) | NS |
Retroperitoneum | 13 (40.6%) | 10 (47.6%) | 2 (22.2%) | 0.193 |
Lungs | 12 (37.5%) | 9 (42.9%) | 2 (22.2%) | NS |
Vasculature | 12 (37.5%) | 10 (47.6%) | 1 (11.1%) | 0.057 |
Central nervous system | 11 (34.4%) | 8 (38.1%) | 3 (33.3%) | NS |
Pericardium | 9 (28.1%) | 8 (38.1%) | 1 (11.1%) | 0.139 |
Pleura | 7 (21.9%) | 7 (33.3%) | 0 (0.0%) | 0.048 |
Skin | 6 (18.8%) | 4 (19.0%) | 2 (22.2%) | NS |
Pituitary | 5 (15.6%) | 4 (19.0%) | 1 (11.1%) | NS |
Heart | 4 (12.5%) | 2 (9.5%) | 1 (11.1%) | NS |
Exophthalmos | 4 (12.5%) | 3 (14.3%) | 1 (11.1%) | NS |
Nerve roots | 3 (9.4%) | 1 (4.8%) | 1 (11.1%) | NS |