Table 1.
Baseline patient and tumor characteristics
| Rhabdomyosarcoma (n=87) | |
|---|---|
| Age at diagnosis | |
| Median (Range) | 16.4 (0.2–74.3) |
|
| |
| Gender | |
| Male | 44 (51%) |
| Female | 43 (49%) |
|
| |
| Histology | |
| Embryonal | 40 (46%) |
| Alveolar | 25 (29%) |
| Spindle cell | 18 (21%) |
| Pleomorphic | 4 (5%) |
|
| |
| PAX3/7-FOXO1 Gene Fusion | |
| Present | 22 (25%) |
| Absent | 65 (75%) |
|
| |
| MYOD1 in spindle cell histology | |
| Mutated | 8 (44%) |
| Wild-type | 10 (56%) |
|
| |
| Primary site | |
| Parameningeal | 24 (28%) |
| Extremity | 20 (23%) |
| GU non-bladder/prostate | 18 (21%) |
| Perineal/perianal | 7 (8%) |
| GU bladder/prostate | 3 (3%) |
| Orbit | 3 (3%) |
| Head and neck | 3 (3%) |
| Other | 9 (10%) |
|
| |
| Stage | |
| 1 | 19 (22%) |
| 2 | 3 (3%) |
| 3 | 38 (44%) |
| 4 | 27 (31%) |
|
| |
| Group | |
| 1 | 11 (13%) |
| 2 | 8 (9%) |
| 3 | 41 (47%) |
| 4 | 27 (31%) |
|
| |
| Risk Group* | |
| Low | 22 (25%) |
| Intermediate | 38 (44%) |
| High | 27 (31%) |
|
| |
| Surgery for primary tumor | |
| Yes | 45 (52%) |
| No | 42 (48%) |
|
| |
| Radiation for primary tumor | |
| Yes | 70 (80%) |
| No | 17 (20%) |
|
| |
| Number of genetic alterations | |
| Fusion-positive tumors, median (range) | 2 (0–8) |
| Fusion-negative tumors, median (range) | 4 (0–25) |
|
| |
| Tumor mutational burden | |
| Fusion-positive tumors, median (range) | 0.9 (0–4.4) |
| Fusion-negative tumors, median (range) | 1.8 (0–13.8) |
*
Risk stratification based on current Children’s Oncology Group risk stratification