Pediatric Rhabdomyosarcoma Incidence and Survival in the United States: An Assessment of 5656 Cases, 2001–2017

Authors

Matthew T McEvoy
David A Siegel
Shifan Dai
Mehmet Fatih Okcu
Mark Zobeck
Rajkumar Venkatramani
Philip J Lupo

Publication Date

2-1-2023

Journal

Cancer Medicine

DOI

10.1002/cam4.5211

PMID

36069287

PMCID

PMC9939205

PubMedCentral® Posted Date

9-7-2022

PubMedCentral® Full Text Version

Post-print

Published Open-Access

yes

Keywords

Adolescent, Humans, Child, United States, Incidence, Rhabdomyosarcoma, Rhabdomyosarcoma, Embryonal, Proportional Hazards Models, Survival Rate, rhabdomyosarcoma, epidemiology, pediatric cancer, incidence, survival, soft tissue sarcoma

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

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.