Patient characteristics
| . | Italian cohort (n = 118) . | French cohort (n = 238) . |
|---|---|---|
| Year of diagnosis | 1996-2022 | 1986-2022 |
| Age at diagnosis, y | 56.5 (19-81) | 62 (21-86) |
| Sex, male | 88 (74.6%) | 168 (70.6%) |
| Somatic mutations | ||
| BRAFV600E | 70 (59.3%) | 121 (50.8%)∗ |
| MAP2K1 | 2 (1.7%) | 10 (4.2%) |
| NRAS | 1 (0.8%) | 1 (0.4%)∗ |
| KRAS | 1 (0.8%) | 4 (1.7%)∗ |
| ERK | 1 (0.8%) | - |
| Wild type or undetermined | 43 (36.4%) | 104 (43.7%) |
| Organ involvement | ||
| Bones | 105 (89.0%) | 192 (80.7%) |
| Perirenal | 81 (68.6%) | 156 (65.5%) |
| Central nervous system | 60 (50.8%) | 86 (36.1%) |
| Pituitary gland | 42 (35.6%) | 58 (24.4%) |
| Heart | 53 (44.9%) | 105 (44.1%) |
| Large vessels | 51 (43.2%) | 143 (60.1%) |
| Skin | 50 (42.4%) | 61 (25.6%) |
| Face | 33 (28.0%) | 78 (32.8%) |
| Lungs | 35 (29.7%) | 79 (33.2%) |
| . | Italian cohort (n = 118) . | French cohort (n = 238) . |
|---|---|---|
| Year of diagnosis | 1996-2022 | 1986-2022 |
| Age at diagnosis, y | 56.5 (19-81) | 62 (21-86) |
| Sex, male | 88 (74.6%) | 168 (70.6%) |
| Somatic mutations | ||
| BRAFV600E | 70 (59.3%) | 121 (50.8%)∗ |
| MAP2K1 | 2 (1.7%) | 10 (4.2%) |
| NRAS | 1 (0.8%) | 1 (0.4%)∗ |
| KRAS | 1 (0.8%) | 4 (1.7%)∗ |
| ERK | 1 (0.8%) | - |
| Wild type or undetermined | 43 (36.4%) | 104 (43.7%) |
| Organ involvement | ||
| Bones | 105 (89.0%) | 192 (80.7%) |
| Perirenal | 81 (68.6%) | 156 (65.5%) |
| Central nervous system | 60 (50.8%) | 86 (36.1%) |
| Pituitary gland | 42 (35.6%) | 58 (24.4%) |
| Heart | 53 (44.9%) | 105 (44.1%) |
| Large vessels | 51 (43.2%) | 143 (60.1%) |
| Skin | 50 (42.4%) | 61 (25.6%) |
| Face | 33 (28.0%) | 78 (32.8%) |
| Lungs | 35 (29.7%) | 79 (33.2%) |
Continuous variables are expressed as median (range). Categorical variables are expressed as n (%).
Some patients harbor 2 mutations.