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Diseases

Genetic and Rare Diseases Information Center (GARD)

Ewing sarcoma


Other Names for this Disease
  • Ewing's tumor
  • Sarcoma, Ewing's
  • Ewing tumor
  • Ewing's sarcoma
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Your Question

What causes Ewing sarcoma? Is it inherited? If a child is affected, are his or her siblings at risk to develop it as well?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is Ewing sarcoma?

Ewing sarcoma is a malignant (cancerous) bone tumor that affects children. It can occur any time during childhood and young adulthood, but usually develops during puberty, when bones are growing rapidly. The tumor may arise anywhere in the body, usually in the long bones of the arms and legs, the pelvis, or the chest. It may also develop in the skull or the flat bones of the trunk. There are few symptoms. The most common is pain and occasionally swelling at the site of the tumor. Fever may also be present. The tumor often spreads (metastasis) to the lungs and other bones.[1] The cause of Ewing sarcoma is unknown. Most cases are thought to occur randomly and many involved a reciprocal translocation between chromosomes 11 and 22. Treatment depends upon a number of factors, but may include chemotherapy, radiation and/or surgical interventions.[2]
Last updated: 2/13/2016

What causes Ewing sarcoma?

The exact cause of Ewing sarcoma remains largely unknown.[2][3] Chromosomal studies have found that Ewing sarcoma cells are often characterized by an abnormal change in their genetic makeup known as a reciprocal translocation.[2][4] The most common mutation, occurring in approximately 85% of Ewing sarcoma tumors, involves two genes, the EWSR1 gene on chromosome 22 and the FLI1 gene on chromosome 11. This rearrangement of genetic material between chromosomes 22 and 11 fuses part of the EWSR1 gene with part of the FLI1 gene, creating the EWSR1/FLI1 fusion gene. This mutation is acquired during a person's lifetime and is present only in tumor cells. This type of genetic change, called a somatic mutation, is not inherited.[4] In extremely rare cases, Ewing sarcoma may develop as a second malignancy, which means that the condition develops as a late-onset complication of earlier treatment for another form of cancer.[2]
Last updated: 2/14/2016

Is Ewing sarcoma an inherited condition?

This condition is generally not inherited but arises from a mutation in the body's cells that occurs after conception (somatic mutation).[4] Most cases are considered to be sporadic.[2][3] However, the incidence of neuroectodermal and stomach malignancies is increased among family members of patients with tumors of the Ewing sarcoma family.[3] 

A search of the medical literature did identify a very small number of cases of Ewing sarcoma among siblings. To access articles on this topic, click here.
Last updated: 2/14/2016

References
Other Names for this Disease
  • Ewing's tumor
  • Sarcoma, Ewing's
  • Ewing tumor
  • Ewing's sarcoma
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.