Approximately 3,000 new bone sarcomas are diagnosed in the United States each year, making malignant bone tumors rare. Ewing sarcoma, a small, round blue cell tumor, is one-third less common than conventional osteosarcoma. Primary, small, round blue cell tumors are composed entirely of cells that have a hematopoietic appearance and without matrix production. Small, round blue cell tumors that affect children and adolescents may be benign or malignant. The most common benign and malignant small, round blue cell tumors are eosinophilic granuloma and Ewing sarcoma, respectively; however, this group encompasses a large number of tumors that appear histologically similar (ie, primary and metastatic lymphoma, metastatic neuroblastoma, multiple myeloma, metastatic small cell carcinoma, and rhabdomyosarcoma). Diagnosis of these tumors heavily relies on clinical presentation and imaging studies; however, histopathologic imaging and staining aid in diagnosis.

Treatment modalities vary greatly among subtypes; however, local tumor control is imperative. Benign entities may be curetted and bone grafted, whereas limb-sparing surgery is preferred for the management of most malignant entities. In patients with Ewing sarcoma, tumor necrosis of more than 90% in response to multiagent preoperative chemotherapy is correlated with a more favorable prognosis. Patients with disseminated disease have a considerably poorer 5-year survival rate. Because of the high potential of this tumor to metastasize, patients with Ewing sarcoma must be monitored closely, treated via chemotherapy and/or radiation, and, if possible, undergo limb-sparing surgery and/or radiation for local control.