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

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) a...

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Main Authors: Matthew T. McEvoy, David A. Siegel, Shifan Dai, Mehmet Fatih Okcu, Mark Zobeck, Rajkumar Venkatramani, Philip J. Lupo
Format: Article
Language:English
Published: Wiley 2023-02-01
Series:Cancer Medicine
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Online Access:https://doi.org/10.1002/cam4.5211
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author Matthew T. McEvoy
David A. Siegel
Shifan Dai
Mehmet Fatih Okcu
Mark Zobeck
Rajkumar Venkatramani
Philip J. Lupo
author_facet Matthew T. McEvoy
David A. Siegel
Shifan Dai
Mehmet Fatih Okcu
Mark Zobeck
Rajkumar Venkatramani
Philip J. Lupo
author_sort Matthew T. McEvoy
collection DOAJ
description 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.
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spelling doaj-art-7af0a496fbc54de58e19d4c233ea5b312025-08-20T02:33:08ZengWileyCancer Medicine2045-76342023-02-011233644365610.1002/cam4.5211Pediatric rhabdomyosarcoma incidence and survival in the United States: An assessment of 5656 cases, 2001–2017Matthew T. McEvoy0David A. Siegel1Shifan Dai2Mehmet Fatih Okcu3Mark Zobeck4Rajkumar Venkatramani5Philip J. Lupo6Texas Children's Cancer Center, Baylor College of Medicine Houston Texas USADivision of Cancer Prevention and Control Centers for Disease Control and Prevention Atlanta Georgia USADivision of Cancer Prevention and Control Centers for Disease Control and Prevention Atlanta Georgia USATexas Children's Cancer Center, Baylor College of Medicine Houston Texas USATexas Children's Cancer Center, Baylor College of Medicine Houston Texas USATexas Children's Cancer Center, Baylor College of Medicine Houston Texas USATexas Children's Cancer Center, Baylor College of Medicine Houston Texas USAAbstract 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.https://doi.org/10.1002/cam4.5211rhabdomyosarcomaepidemiologypediatric cancerincidencesurvivalsoft tissue sarcoma
spellingShingle Matthew T. McEvoy
David A. Siegel
Shifan Dai
Mehmet Fatih Okcu
Mark Zobeck
Rajkumar Venkatramani
Philip J. Lupo
Pediatric rhabdomyosarcoma incidence and survival in the United States: An assessment of 5656 cases, 2001–2017
Cancer Medicine
rhabdomyosarcoma
epidemiology
pediatric cancer
incidence
survival
soft tissue sarcoma
title Pediatric rhabdomyosarcoma incidence and survival in the United States: An assessment of 5656 cases, 2001–2017
title_full Pediatric rhabdomyosarcoma incidence and survival in the United States: An assessment of 5656 cases, 2001–2017
title_fullStr Pediatric rhabdomyosarcoma incidence and survival in the United States: An assessment of 5656 cases, 2001–2017
title_full_unstemmed Pediatric rhabdomyosarcoma incidence and survival in the United States: An assessment of 5656 cases, 2001–2017
title_short Pediatric rhabdomyosarcoma incidence and survival in the United States: An assessment of 5656 cases, 2001–2017
title_sort pediatric rhabdomyosarcoma incidence and survival in the united states an assessment of 5656 cases 2001 2017
topic rhabdomyosarcoma
epidemiology
pediatric cancer
incidence
survival
soft tissue sarcoma
url https://doi.org/10.1002/cam4.5211
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