Abstract
BackgroundThe purpose of this study was to develop a prognostic model for the survival of pediatric patients with rhabdomyosarcoma (RMS) using parameters that are measured during routine clinical management.MethodsDemographic and clinical variables were evaluated in 1679 pediatric patients with RMS registered in the Surveillance, Epidemiology, and End Results (SEER) program from 1990 to 2010. A multivariate Cox proportional hazards model was developed to predict median, 5-year and 10-year overall survival (OS). The Akaike information criterion technique was used for model selection. A nomogram was constructed using the reduced model after model selection, and was internally validated.ResultsOf the total 1679 patients, 543 died. The 5-year OS rate was 64.5% (95% confidence interval (CI), 62.1-67.1%) and the 10-year OS was 61.8% (95%CI, 59.2-64.5%) for the entire cohort. Multivariate analysis identified age at diagnosis, tumor size, histological type, tumor stage, surgery and radiotherapy as significantly associated with survival (p < 0.05). The bootstrap-corrected c-index for the model was 0.74. The calibration curve suggested that the model was well calibrated for all predictions.ConclusionsThis study provided an objective analysis of all currently available data for pediatric RMS from the SEER cancer registry. A nomogram based on parameters that are measured on a routine basis was developed. The nomogram can be used to predict 5- and 10-year OS with reasonable accuracy. This information will be useful for estimating prognosis and in guiding treatment selection.
Highlights
The purpose of this study was to develop a prognostic model for the survival of pediatric patients with rhabdomyosarcoma (RMS) using parameters that are measured during routine clinical management
Study population The data were derived from the SEER program, which collects demographic, diagnostic and treatment information on all newly diagnosed cancer patients residing within specific US geographic regions
Registry data are submitted without personal identifiers to the National Cancer Institute (NCI), and these data are publicly available for research purpose
Summary
The purpose of this study was to develop a prognostic model for the survival of pediatric patients with rhabdomyosarcoma (RMS) using parameters that are measured during routine clinical management. Rhabdomyosarcoma (RMS) is the most common softtissue sarcoma in children and adolescents and accounts for 3% of all pediatric tumors [1]. 350 children are diagnosed with RMS in the United States every year [2]. Incidence peaks at a very young age. Because RMS is derived from immature striated skeletal muscle, this disease can occur at any site in the body. Prognosis of RMS has improved significantly, with multidisciplinary management accounting for most of the increase in survival rate. Since 1972, the Intergroup Rhabdomyosarcoma Study Group (IRSG) has conducted
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