No.(ratio %) | |
---|---|
Total patient | 36 |
Gender | |
Male | 21 (58.3%) |
Female | 15 (41.7%) |
Age at diagnosis (yrs) | |
Median (range) | 35.5 (18–57) |
Disease classification | |
Multifocal single system (SS-m) | 5 (13.9%) |
Multiple system without risk organ involvement (RO-) | 20 (55.6%) |
Multiple system with risk organ involvement (RO+) | 11 (30.6%) |
Liver involvement | 10 (27.8%) |
Spleen involvement | 1 (2.8%) |
Bone marrow (BM) involvement | 1 (2.8%) |
Other organ/system involvement | |
Pulmonary involvement |
21 (58.3%) 20/21 manifesting with pulmonary function disturbance. |
Bone involvement | 31 (86.1%) |
Bone aching | 18 (50%) |
Lytic lesions | 31 (86.1%) |
Lesion sites | |
Skull bone | 22 (61.1%) |
Vertebra | 18 (50%) |
Pelvis | 13 (36.1%) |
Ribs | 11 (30.6%) |
Extremity bones | 8 (22.2%) |
Central nervous system involvement | 25 (69.4%) |
Pituitary | 23 (63.9%) |
Other sitesa | 2 (5.6%) |
Lymph nodes involvement | 21 (58.3%) |
CNS-risk organ involvement | 8 (22.2%) |
Thyroid involvement | 7 (19.4%) |
BRAFV600E mutation | |
No. of patients tested | 19 |
No. of positivity | 4 (21.1%) |