Prognostic factors of desmoplastic small round cell tumor in children and adolescents: a population-based study

PurposeDesmoplastic small round cell tumor (DSRCT) in children is extremely rare and aggressive. We aimed to conduct a population-based cohort study to predict overall survival (OS) in pediatric patients with DSRCT.MethodsUsing the Surveillance, Epidemiology and End Results (SEER) database, all pediatric patients diagnosed with DSRCT between 2000 and 2018 were located. Kaplan-Meier estimates were used to generate survival curves based on different criteria. The survival curves were compared using the log-rank test. Cox proportional-hazards regression was used to identify the variables related to overall survival (OS).ResultsA total of 129 pediatric DSRCT patients were identified. The tumors primarily affected males (70.5%) and distant metastasis was present in 65.9% of all cases. Most of them received surgery (61.3%). Chemotherapy and radiotherapy were utilized in 90% and 41.8% of cases, respectively. Overall 1-year, 3-year, and 5-year survival rates for all patients were 81.9%, 37.9%, and 22.6%, respectively. Patients with distant stage showed a greater risk of mortality (hazard ratio (HR) 4.75, 95% confidence interval (CI) 1.87-12.0; P = 0.001). In addition, patients who received radiotherapy had a lower risk of mortality (hazard ratio (HR) 0.45, 95% confidence interval (CI) 0.25-0.81; P = 0.008).ConclusionsDSRCT is a rare malignancy with a generally poor prognosis. Our findings demonstrated that SEER stage and radiotherapy were significant predictors of OS in pediatric DSRCT. Radiotherapy has the potential to improve the prognosis for pediatric patients.