TABLE 4.
No. (%) | |||||
---|---|---|---|---|---|
Characteristic | Low risk, N = 176 | Medium risk, N = 701 | High risk, N = 423 | Very high risk, N = 361 | |
Age at diagnosis | Birth to 3 years | 6 (3) | 256 (36) | 23 (5) | 182 (50) |
3–10 years | 102 (58) | 277 (40) | 331 (78) | 61 (17) | |
10 years and older | 68 (39) | 168 (24) | 69 (17) | 118 (33) | |
Tumor size | ≤5 cm | 147 (83) | 438 (62) | 144 (34) | 86 (24) |
>5 cm | 29 (17) | 263 (38) | 279 (66) | 275 (76) | |
Primary site | Extremities/HNPM/other sites | — | 80 (11) | 346 (82) | 353 (98) |
GUBP/HNnoPM/orbit | 21 (12) | 451 (64) | 54 (13) | 8 (2) | |
GUnoBP/bile ducts | 155 (88) | 170 (24) | 23 (5) | — | |
IRS group | I | 128 (73) | 73 (10) | 3 (1) | — |
II | 48 (27) | 142 (20) | 12 (3) | 2 (1) | |
III | — | 486 (69) | 408 (96) | 359 (99) | |
Fusion status | Negative | 176 (100) | 661 (94) | 360 (85) | 196 (54) |
Positive | 40 (6) | 63 (15) | 165 (46) | ||
Age/tumor size | Birth to 3 years/>5 cm | 6 (3) | 112 (16) | 3 (1) | 113 (31) |
Birth to 3 years/≤5 cm | — | 144 (20) | 20 (5) | 69 (19) | |
3–10 years/≤5 cm | 79 (45) | 194 (28) | 104 (25) | 17 (5) | |
3–10 years/>5 cm | 23 (13) | 83 (12) | 227 (54) | 44 (12) | |
10 years and older/≤5 cm | 68 (39) | 100 (14) | 20 (5) | — | |
10 years and older/>5 cm | — | 68 (10) | 49 (12) | 118 (33) | |
EFS probability, % | 3 years [95% CI] | 94.1 [89.4–96.8] | 80.6 [77.4–83.3] | 68.4 [63.7–72.6] | 54.3 [48.9–59.3] |
5 years [95% CI] | 94.1 [89.4–96.8] | 78.4 [75.1–81.3] | 65.2 [60.4–69.6] | 52.1 [46.8–57.2] | |
Type of event | Dead | — | 2 (1) | 3 (2) | — |
Local‐regional | 6 (55) | 96 (64) | 66 (44) | 77 (43) | |
Metastases progression | 4 (36) | 24 (16) | 40 (26) | 60 (34) | |
Other | 1 (9) | 10 (7) | 13 (9) | 5 (3) | |
Progressive disease | — | 19 (13) | 29 (19) | 36 (20) | |
OS probability | 3 years [95% CI] | 98.8 [95.2–99.7] | 94.0 [92.0–95.6] | 78.7 [74.5–82.4] | 72.5 [67.5–76.8] |
5 years [95% CI] | 97.2 [92.7–99.0] | 91.5 [89.0–93.4] | 74.3 [69.7–78.3] | 60.8 [55.4–65.9] |
Abbreviations: CI, confidence interval; EFS, event‐free survival; GUBP, genitourinary bladder‐prostate; HNnoPM, head and neck nonparameningeal; HNPM, head and neck parameningeal; IRS, Intergroup Rhabdomyosarcoma Studies; OS, overall survival.