Survival stratification in childhood rhabdomyosarcoma of the extremities: a derivation and validation study

Sci Rep. 2020 Mar 30;10(1):5684. doi: 10.1038/s41598-020-62656-x.


The objective of this study was to estimate overall survival in children with extremity rhabdomyosarcoma (RMS). In addition, we attempted to construct a nomogram to predict the prognosis in such patients using a population-based cohort. The national Surveillance, Epidemiology, and End Results (SEER) registry was used to identify a cohort of childhood RMS patients. A total of 197 patients with RMS were ultimately included. Multivariable analysis identified age group, N classification, M classification, and treatment combinations as independent predictive factors for patient overall survival. Candidate variables such as age group, N classification, M classification, and treatment combinations were used to fit the model. For overall survival, the bootstrap-adjusted c-index was 0.76 (95% CI, 0.73-0.80) for the nomogram. Furthermore, we performed recursive partitioning analysis for risk stratification according to overall survival, and 3 prognostic subgroups were generated (low, intermediate and high risk). Finally, we evaluated multimodal treatment based on the risk stratification according to the nomogram and IRSG prognostic stratification model. With regard to the entire cohort, overall survival in patients who received surgery and radiation was superior to that in patients who received surgery or radiation (p = 0.001). Regarding RPA and IRSG prognostic stratification, we found that the differences remained significant (p < 0.05) in patients with low-intermediate risk. However, the difference disappeared in patients with high risk (p > 0.05). We performed a population-based analysis of data from the SEER registry in an effort to identify prognostic factors and develop a nomogram in children with extremity RMS. The nomogram appears to be suitable for the survival stratification of children with RMS and will help clinicians identify patients who may be at a reduced probability of survival and assist them in making treatment and surveillance decisions. More studies concerning overall survival in children with RMS are needed to confirm and update our findings.

MeSH terms

  • Adolescent
  • Child
  • Child, Preschool
  • Cohort Studies
  • Combined Modality Therapy / methods
  • Extremities / physiopathology
  • Female
  • Humans
  • Infant
  • Male
  • Nomograms
  • Probability
  • Prognosis
  • Rhabdomyosarcoma / mortality*
  • Rhabdomyosarcoma / therapy*
  • Risk Factors
  • SEER Program