Ewings Sarcoma

Ewing sarcoma family of tumors includes classically described Ewing sarcoma (ES), peripheral primitive neuroectodermal tumor (PNET), Askin-Rosai tumor of the chest wall and extraskeletal Ewing sarcoma. Ewing sarcoma is the second most common bone malignancy after osteosarcoma. These tumors are characterized by a relatively simple karyotype, most commonly the ews gene rearrangements on chromosome 22, with a clinical predilection for presentation in the adolescent age group, diaphyseal location and bone destruction. Management is multimodal with chemotherapy, radiotherapy and surgery based on multidisciplinary principles. With dose-dense chemotherapy and optimal local therapy, long-term survivals have improved significantly in the last few decades. Focus on newer modalities of therapies, nutritional and psychosocial support, age-appropriate care of adolescent survivors for rehabilitation back into being productive members of society, is necessary for optimal care of patients with Ewing sarcoma.