Ewing's sarcoma

Last edited 01/2021 and last reviewed 03/2021

Ewing's sarcoma is a malignant small cell tumour of bone, more common in males and having its highest incidence in the age group 5 to 20 years. It usually occurs in the diaphysis of long bones.

Ewing's sarcomas account for about 2% of paediatric malignancies:

• Ewing's sarcoma arises predominantly in children and young adults, with an incidence of 1 case per 1.5 million population, a frequency among persons of European ancestry that is almost 10 times as high as the frequency among those of African ancestry, a slight predilection for males (ratio of cases among males to cases among females, 1.6:1.0), and a peak incidence at 15 years of age
• is the second most common bone cancer in children, and can occur in any part of the body but most commonly involves the pelvis and proximal long bones
• most often occurs in the femur, humerus, tibia, pelvis and ribs. Metastasis occurs early, usually to the lungs and other bones.
• in approximately 20% of patients, tumors are extraosseous and can arise in numerous organs; extraosseous Ewing's sarcoma occurs much more frequently in adults than in children

Ewing's sarcoma may mimic an infective process and present with localised swelling, redness, heat and fever. There may be a raised WBC and ESR. Biopsy is needed for diagnosis.

• most important prognostic factor is the presence of metastasis at the time of diagnosis
• patients with local disease that responds to multimodal therapy currently have a 5-year survival rate of more than 70%
• less than 30% of patients presenting with metastases survive for 5 years

Reference:

• Riggi N et al. Ewing’s Sarcoma. N Engl J Med 2021;384:154-64.DOI: 10.1056/NEJMra2028910