Ewing Sarcoma/Peripheral Neuroectodermal Tumor of Bone and Soft Tissue in Infants: A Report from Children Cancer Hospital of Egypt

Abstract

Introduction: Ewing sarcoma is an aggressive malignancy of bone and soft tissue. It is rare in children under age 5years. Ewing's sarcoma family tumors include classic Ewing's sarcoma, primitive neuroectodermal tumour and Askin tumor. ES is the second most common variety of primary bone cancer in adolescents and young adults .The treatment strategy for ES is characterized by multi-disciplinary collaboration between pediatric oncologists, radiation oncologists, and orthopedic surgeons. Although the survival rate of ES patients has improved, their prognosis remains unsatisfactory.The treatment of ES is still challenging specially in age group below 3 years. Patients and methods: A Retrospective study of children below 3 years with a diagnosis Ewing Sarcoma/PNET presented to CCHE from July 2009 till December 2018. Results: A review of 46 patients with age ranged from 3 months to 3 years with Mean of age was 1.8. Primary site was Osseous and extraosseus in 23 infant patients for each. Main site was mainly axial in 28 cases (60.8%). Cases were treated according to COG protocol. Infantile Ewing sarcoma OS and EFS at 5-years OS were 75.5% and 66.7%. Conclusion: Patients below 3 years represent an unusual young age group. Conclusion: ES/PNET in age below 3 years is a unique age group with a different management and outcome.