TABLE 2.
Five‐year relative survival (RS) and univariate all‐cause survival hazard ratios (HR) for children and adolescents with rhabdomyosarcoma in the national program of cancer registries, 2001–2016
| Relative survival | All‐cause survival | |||||
|---|---|---|---|---|---|---|
| Variable | No. (%) | 5‐year RS (%) | 95% CI (%) | HR | 95% CI | p value |
| Total | 5589 (100) | 68.0 | 66.6–69.3 | |||
| Sex | ||||||
| Male | 3228 (58) | 70.4 | 68.7–72.1 | Ref | ||
| Female | 2361 (42) | 64.7 | 62.5–66.7 | 1.25 | 1.13–1.38 | <0.0001 |
| Age at diagnosis (years) | ||||||
| <1 | 317 (6) | 70.6 | 64.8–75.7 | 1.45 | 1.16–1.82 | 0.001 |
| 1–9 | 3094 (55) | 77.2 | 75.5–78.7 | Ref | ||
| 10–19 | 2178 (39) | 54.4 | 52.1–56.6 | 2.30 | 2.07–2.55 | <0.0001 |
| Race/Ethnicity | ||||||
| NH White | 3269 (58) | 69.3 | 67.6–71.0 | Ref | ||
| NH Black | 941 (17) | 64.4 | 61.0–67.6 | 1.20 | 1.05–1.36 | 0.01 |
| NH API | 196 (4) | 64.8 | 56.7–71.8 | — | — | — |
| NH AI/AN | 53 (1) | 69.9 | 53.3–81.5 | — | — | — |
| Hispanic | 1130 (20) | 67.5 | 64.5–70.4 | 1.09 | 0.96–1.24 | 0.18 |
| Histology (ICD‐O‐3 code) | ||||||
| Embryonal (ERMS) | 2802 (50) | 79.2 | 77.5–80.7 | Ref | ||
| Spindle | 197 (4) | 85.4 | 79.0–90.0 | 0.66 | 0.44–0.99 | 0.04 |
| Alveolar (ARMS) | 1653 (30) | 47.9 | 45.2–50.5 | 2.96 | 2.64–3.31 | <0.0001 |
| Others a | 937 (17) | 66.9 | 63.5–70.0 | 1.76 | 1.52–2.04 | <0.0001 |
| Primary tumor site | ||||||
| Favorable | ||||||
| Orbit | 364 (7) | 93.4 | 90.0–95.7 | Ref | ||
| Genitourinary system | 711 (13) | 87.4 | 84.5–89.8 | 2.07 | 1.28–3.34 | 0.003 |
| Biliary tract/liver | 230 (4) | 82.8 | 76.8–87.4 | 3.05 | 1.77–5.23 | <0.0001 |
| Head/neck | 1066 (19) | 71.9 | 68.8–74.7 | 5.00 | 3.20–7.80 | <0.0001 |
| Unfavorable | ||||||
| Bladder/prostate | 326 (6) | 74.4 | 68.9–79.1 | 4.44 | 2.74–7.20 | <0.0001 |
| Para‐meningeal region | 493 (9) | 62.8 | 58.1–67.1 | 6.93 | 4.40–10.90 | <0.0001 |
| Extremity | 744 (13) | 54.7 | 50.7–58.6 | 8.62 | 5.54–13.43 | <0.0001 |
| Trunk/other | 1633 (29) | 55.4 | 52.7–58.0 | 8.96 | 5.80–13.84 | <0.0001 |
| Unknown | 22 (<1) | 36.4 | 17.4–55.7 | N/A | — | — |
| Stage | ||||||
| Localized | 1892 (34) | 87.9 | 86.2–89.4 | Ref | ||
| Regional | 1801 (32) | 73.0 | 70.7–75.1 | 2.45 | 2.07–2.90 | <0.0001 |
| Distant | 1575 (28) | 38.5 | 35.9–41.1 | 7.55 | 6.46–8.82 | <0.0001 |
| Unknown | 321 (6) | 66.9 | 61.0–72.0 | N/A | — | — |
| US Census region | ||||||
| Northeast | 988 (18) | 68.6 | 65.4–71.6 | Ref | ||
| Midwest | 1060 (19) | 69.9 | 66.9–72.8 | 0.95 | 0.81–1.13 | 0.57 |
| South | 2183 (39) | 67.6 | 65.4–69.7 | 1.03 | 0.90–1.19 | 0.66 |
| West | 1358 (24) | 66.6 | 63.8–69.2 | 1.08 | 0.93–1.26 | 0.32 |
| Socioeconomic status | ||||||
| Top 25% | 1480 (26) | 69.6 | 66.9–72.1 | Ref | ||
| 25%–75% | 3283 (59) | 67.6 | 65.8–69.3 | 1.12 | 0.99–1.26 | 0.07 |
| Bottom 25% | 716 (13) | 65.5 | 61.6–69.1 | 1.17 | 0.99–1.38 | 0.07 |
| Unknown | 110 (2) | 74.2 | 64.1–81.8 | 0.83 | 0.56–1.25 | 0.37 |
| Population density by county | ||||||
| Metro (population) | 4884 (87) | 67.9 | 66.4–69.3 | |||
| >1,000,000 | 3299 (59) | 68.4 | 66.7–70.1 | Ref | ||
| 250,000‐1,000,000 | 1119 (20) | 66.9 | 63.8–69.8 | 1.08 | 0.95–1.22 | 0.26 |
| <250,000 | 466 (8) | 66.1 | 61.3–70.5 | 1.13 | 0.95–1.35 | 0.17 |
| Non‐Metro | 704 (13) | 68.7 | 64.9–72.2 | 1.00 | 0.86–1.17 | 0.99 |
| Year of diagnosis | ||||||
| 2001–2005 | 1712 (31) | 67.4 | 65.1–69.5 | Ref | ||
| 2006–2010 | 1804 (32) | 67.9 | 65.7–70.0 | 0.96 | 0.86–1.08 | 0.53 |
| 2011–2016 | 2073 (37) | 68.6 | 65.7–71.2 | 0.94 | 0.83–1.06 | 0.31 |
Note: Dash indicates unable to calculate due to sampling size/missing data.
Abbreviations: AI/AN, American Indian/Alaska Native; API, Asian‐Pacific Islander; CI, confidence interval; HR, Cox hazard ratio; ICD, International Classification of Diseases; NH, non‐Hispanic; RS, relative survival; SEER, surveillance, epidemiology, and end results.
Other histologies include RMS NOS, mixed‐type RMS, and embryonal sarcoma. Patients with NH unknown race have been excluded from this analysis (n = 63). Variables with missing individual cases (n): population density (1).