Ewing's sarcoma: current status of diagnosis and treatment

Ewing's sarcoma, second only to osteosarcoma as the most common malignant bone tumor of children and adolescents, can be cured in more than half of all cases. Combination chemotherapy and local irradiation is the most effective multimodality approach, with chemotherapy being initiated as soon as possible after diagnosis and work-up. The first Intergroup Ewing's Sarcoma Study, completed in 1978, demonstrated that adjuvant therapy with vincristine, dactinomycin was superior to VAC alone or VAC with pulmonary irradiation. The most recent treatment advance is the identification of a high level of activity of ifosfamide and etoposide in patients with recurrent disease. This combination will soon be tested in a collaborative group Phase III trial.