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

Abstract

Purpose: Desmoplastic 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.

Methods: Using 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).

Results: A 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).

Conclusions: DSRCT 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.