Demographic characteristics and prognostic factors in pediatric-type sarcomas; A 7 year single institutional experience and comprehensive review of the current literature

Background: Due to limited clinical data in pediatric-type sarcomas (rhabdomyosarcoma, Ewing's sarcoma, PNET, and desmoplastic small round-cell tumor), the aim of this study was to evaluate the demographic characteristics and identifying prognostic factors for survival. Materials and Methods: We retrospectively reviewed 110 patients with pediatric-type sarcomas. Overall and disease free survival was analyzed with the Kaplan-Meier method and log rank test. To identify prognostic factors for overall and disease free survival, multivariate survival analyses using a Cox’s proportional-hazard regression model was performed. Results: In this study mean age of patients were 20.30 years (SD=13.61; range, 1–83 years). The survival data of 54 patients (49.1%) were obtained with median survival of 27 months. 3 and 5-year survival rate of these patients were 41.5% and 28.3% respectively. Recurrence of disease (P=0.006) and Ewing sarcoma subtype (P=0.018) were significantly associated with poor overall survival and location of the lesion in the upper extremities (P=0.007) and trunk (P=0.005) were significantly associated with a lower disease free survival. Conclusion: With multivariate analysis, the authors determined that recurrence of disease and Ewing's sarcoma subtype are poor prognostic factors for overall survival and site of origin for disease free survival among patients with pediatric-type sarcoma. In addition, gender, patient's age, and size of tumor had no significant impact on overall and disease free survival.