Prognostic Factors in Patients with Rhabdomyosarcoma Using Competing-Risks Analysis: A Study of Cases in the SEER Database

Abstract

Background. Rhabdomyosarcoma (RMS) is a rare malignant soft-tissue sarcoma characterized by a poor outcome and unclear prognostic factors. This study applied a competing-risks analysis using data from the Surveillance, Epidemiology, and End Results (SEER) database to RMS patients, with the aim of identifying more accurate prognostic factors. Methods. Data of all patients with RMS during 1986–2015 were extracted from the SEER database. We used the competing-risks approach to calculate the cumulative incidence function (CIF) for death due to rhabdomyosarcoma (DTR) and death from other causes (DOC) at each time point. The Fine–Gray subdistribution proportional-hazards model was then applied in univariate and multivariate analyses to determine how the CIF differs between groups and to identify independent prognostic factors. The potential prognostic factors were analyzed using the competing-risks analysis methods in SAS and R statistical software. Results. This study included 3399 patients with RMS. The 5-year cumulative incidence rates of DTR and DOC after an RMS diagnosis were 39.9% and 8.7%, respectively. The multivariate analysis indicated that age, year of diagnosis, race, primary site, historic stage, tumor size, histology subtype, and surgery status significantly affected the probability of DTR and were independent prognostic factors in patients with RMS. A nomogram model was constructed based on multivariate models for DTR and DOC. The performances of the two models were validated by calibration and discrimination, with C-index values of 0.758 and 0.670, respectively. Conclusions. A prognostic nomogram model based on the competing-risks model has been established for predicting the probability of death in patients with RMS. This validated prognostic model may be useful when choosing treatment strategies and for predicting survival.