TABLE 3.
Specific cardiac and non-cardiac involvements of Erdheim–Chester patients according to different genotypes.
| NRAS | KRAS | BRAF | MAP2K1 | PIK3CA | Other | P value | |
|
|
|||||||
| n = 9 | n = 6 | n = 273 | n = 11 | n = 3 | n = 8 | ||
| Coated aorta, n (%) | 5 | 0 | 84 | 5 | 1 | 1 | ns |
| (55.6) | (0.0) | (32.3) | (45.5) | (33.3) | (12.5) | ||
| Peri-myocardial formationsa, n (%) | 1 | 1 | 65 | 1 | 0 | 1 | ns |
| (11.1) | (16.7) | (24.6) | (9.1) | (0.0) | (12.5) | ||
| Pericardial involvement, n (%) | 0 | 2 | 51 | 1 | 0 | 2 | ns |
| (0.0) | (33.3) | (19.6) | (9.1) | (0.0) | (25.0) | ||
| Cerebral parenchymal involvement, n (%) | 2 | 0 | 115 | 1 | 1 | 0 | 0.008 |
| (22.2) | (0.0) | (42.1) * | (9.1) * | (33.3) | (0.0) | ||
| Pituitary involvement, n (%) | 0 | 0 | 91 | 3 | 0 | 0 | 0.009 |
| (0.0) | (0.0) | (33.3) * | (27.3) | (0.0) | (0.0) | ||
| Retro-orbital infiltration, n (%) | 1 | 0 | 52 | 1 | 1 | 2 | ns |
| (11.1) | (0.0) | (19) | (9.1) | (33.3) | (25.0) | ||
| Pleural involvement, n (%) | 4 | 2 | 25 | 1 | 0 | 3 | 0.002 |
| (44.4) * | (33.3) | (9.2) * | (9.1) | (0.0) | (37.5) | ||
| Peritoneal involvement, n (%) | 2 | 0 | 24 | 4 | 0 | 2 | 0.01 |
| (22.2) | (0.0) | (8.8) * | (8.8) * | (0.0) | (25.0) | ||
| Xanthelasma lesions, n (%) | 3 | 3 | 38 | 0 | 0 | 4 | 0.005 |
| (33.3) | (50.0) * | (14.6) | (0.0) | (0.0) | (50.0) * | ||
| Papulonodular lesions, n (%) | 0 | 3 | 24 | 2 | 0 | 1 | 0.01 |
| (0.0) | (50.0) * | (9.2) | (18.2) | (0.0) | (12.5) | ||
aPresence of at least one between myocardial, right atrium and atrio-ventricular junction infiltration.
*Categories for which there is a statistically significant difference. Statistically significant p-values are in bold.