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. 2023 Feb;12(3):3644-3656.
doi: 10.1002/cam4.5211. Epub 2022 Sep 7.

Pediatric rhabdomyosarcoma incidence and survival in the United States: An assessment of 5656 cases, 2001-2017

Matthew T McEvoy  1 , David A Siegel  2 , Shifan Dai  2   3 , Mehmet Fatih Okcu  1 , Mark Zobeck  1 , Rajkumar Venkatramani  1 , Philip J Lupo  1
Affiliations

Pediatric rhabdomyosarcoma incidence and survival in the United States: An assessment of 5656 cases, 2001-2017

Matthew T McEvoy et al. Cancer Med. 2023 Feb.

Abstract

Background: While rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children and adolescents, past epidemiology studies of this malignancy used data that covered <30% of the US population. Therefore, we evaluated RMS incidence using data from U.S. Cancer Statistics (USCS) and survival trends using the National Program of Cancer Registries (NPCR), which covers 100% and 94% of the U.S. population, respectively.

Methods: Incidence and survival were assessed for pediatric patients diagnosed with RMS during 2003-2017 and 2001-2016, respectively. Both demographic and clinical variables were evaluated. Age-adjusted incidence rates, average annual percent change (AAPC), and 5-year relative survival (RS) were calculated, all with corresponding 95% confidence intervals (CIs). Cox regression models were used to evaluate the impact of demographic and clinical variables on survival.

Results: We identified 5656 primary RMS cases in USCS during 2003-2017. The age-adjusted incidence rate was 4.58 per 1 million (95% CI: 4.46-4.70) with an AAPC of 0.3% (95% CI: -0.7 to 1.2%). In NPCR, 5-year RS for all cases was 68.0% (95% CI: 66.6-69.3%). In multivariable analyses, non-Hispanic (NH) Black cases had worse survival compared with NH White cases (hazard ratio [HR] = 1.16, 95% CI: 1.01-1.33).

Conclusion: The incidence and survival rates were stable in the largest and most comprehensive population-based analysis for pediatric RMS cases in the U.S. Additionally, we observed a survival disparity among NH Black cases. Findings from this study could inform interventions to address disparities, risk stratification strategies, and clinical trial design.

Keywords: epidemiology; incidence; pediatric cancer; rhabdomyosarcoma; soft tissue sarcoma; survival.

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Figures

FIGURE 1
FIGURE 1
Relative survival for children and adolescents with rhabdomyosarcoma by year of diagnosis for sex, histology, and stage.
FIGURE 2
FIGURE 2
Kaplan–Meier survival estimation curve for children and adolescents with rhabdomyosarcoma by (A) sex, (B) age, (C) race/ethnicity, and (D) socioeconomic status.
FIGURE 3
FIGURE 3
Multivariable Cox regression analysis with adjusted hazard ratios (aHR) for 5‐year all‐cause survival in children and adolescents with rhabdomyosarcoma in the National Program of Cancer Registries, 2001–2016. *Patients with NH Asian‐Pacific Islander and NH American Indian/Alaska Native race are combined into a single variable (NH Other). NH Unknown race has been excluded from this analysis (n = 63). ^Other histologies include RMS NOS, mixed‐type RMS, and embryonal sarcoma. aHR, adjusted hazard ratio; NH, non‐Hispanic; SEER, Surveillance, Epidemiology, and End Results.
See this image and copyright information in PMC

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