Extraosseous Ewing Sarcoma in Children: A Systematic Review and Meta-Analysis of Clinicodemographic Characteristics

Abstract

Background: We conducted this systematic review to provide comprehensive evidence on the prevalence, clinical features and outcomes of young extraosseous Ewing sarcoma (EES) cases. Methods: PubMed, Scopus, Web of Science, and Google Scholar were searched for articles reporting the occurrence of EES among children and adolescents (Results: A total of 29 studies were included. Twenty-four reported instances of childhood disease among all the EES cases [ES = 30%; 95%CI: 29–31%], while five studies reported extraosseous cases among the pediatric EES cases [ES = 22%; 95%CI: 13–31%]. The thorax is the most common location of childhood EES [33%; 95%CI: 20–46%] followed by the extremities [31%; 95%CI: 22–40%]. Concurrent chemotherapy and radiotherapy [57%; 95%CI: 25–84%] was the most commonly implemented management protocol in the pediatric EES cases. The rate of no evidence of disease and 5-year overall survival was 69% for both outcomes. Mortality occurred in 29% of cases, while recurrence and secondary metastasis occurred in 35% and 16% of cases, respectively. Conclusions: Our findings provide insight into the clinical features and outcomes of EES among children and adolescents.

Keywords

• Ewing sarcoma
• extraosseous
• children