(iii) Ewing's sarcoma of bone

Ewing's sarcoma is a primitive malignant bone tumour consisting of small, blue, round malignant cells that may show varying degrees of neural differentiation. It accounts for approximately 5% of all malignant bone tumours and arises most frequently in children or adolescents. Despite significant progress with the use of intensive multiagent chemotherapy and local control measures (survival rates have increased from 10-15% to 65-70% in the last 40 years), a significant proportion of patients still die of disease progression. Clinical and biological prognostic factors should be used to guide the therapeutic choices for each patient. The treatment of Ewing's sarcoma of bone is currently based on combined therapy with neoadjuvant chemotherapy, radiation therapy and surgical resection of the primary tumour. Survival in primary metastatic Ewing's sarcoma is poor (less than 30%) and new therapies are needed for these patients.