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

Abstract

Background: Due to limited clinical data in pediatric-type sarcomas (rhabdomyosarcoma, Ewing's sarcoma, PNET, and desmoplas c small round-cell tumor), the aim of this study was to evaluate the demographic characteris cs and iden fying prognos c factors for survival. Materials and Methods: We retrospec vely reviewed 110 pa ents with pediatric-type sarcomas. Overall and disease free survival was analyzed with the Kaplan-Meier method and log rank test. To iden fy prognos c factors for overall and disease free survival, mul variate survival analyses using a Cox’s propor onal-hazard regression model was performed. Results: In this study mean age of pa ents were 20.30 years (SD=13.61; range, 1–83 years). The survival data of 54 pa ents (49.1%) were obtained with median survival of 27 months. 3 and 5-year survival rate of these pa ents were 41.5% and 28.3% respec vely. Recurrence of disease (P=0.006) and Ewing sarcoma subtype (P=0.018) were significantly associated with poor overall survival and loca on of the lesion in the upper extremi es (P=0.007) and trunk (P=0.005) were significantly associated with a lower disease free survival. Conclusion: With mul variate analysis, the authors determined that recurrence of disease and Ewing's sarcoma subtype are poor prognos c factors for overall survival and site of origin for disease free survival among pa ents with pediatric-type sarcoma. In addi on, gender, pa ent's age, and size of tumor had no significant impact on overall and disease free survival.