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Ewing sarcoma is a cancer that most commonly occurs in adolescents, teens, and young adults. The exact cause of most cases is not known.

What causes Ewing sarcoma?

Certain gene changes are seen in Ewing sarcoma cells. These changes cause cells to grow and divide abnormally and lead to cancer.

Gene changes in Ewing sarcoma cells

DNA is the chemical in each of our cells that makes up our genes, which control how our cells function. Changes (mutations) in the DNA inside our cells can sometimes alter the way some genes work, which can cause cells to grow out of control and become cancer.

Some genes control when our cells grow, divide into new cells, and die:

• Genes that normally help cells grow, divide, or stay alive can sometimes lose the ability to turn off and become oncogenes.
• Genes that tell cells to stop dividing or cause cells to die at the right time are called tumor suppressor genes.
• DNA repair genes find and fix damage to the DNA that might happen during cell growth or from the environment.

Researchers have found chromosome changes that lead to Ewing sarcoma, but these changes are not inherited. Instead, they develop in a single cell after a child is born, for unknown reasons.

Nearly all Ewing sarcoma cells have changes that involve the EWSR1 gene, which is found on chromosome 22. Less commonly, changes in the FUS gene on chromosome 16 can be found in this type of cancer. Activation of these genes leads to overgrowth of the cells and to the development of Ewing sarcoma, but the exact way in which this happens is not yet clear.

Risk factors for Ewing sarcoma

A risk factor is anything that affects a person’s chance of getting a disease, such as cancer. Risk factors for developing Ewing sarcoma are not known.

While the gene changes that lead to Ewing tumors are known, it’s still not clear what causes these changes. They might just be random events that sometimes happen inside a cell, without having an outside cause.

In some cases, people with certain gene changes before birth or those passed along in the family can have a higher risk of certain cancers, but this does not seem to be the case for Ewing sarcoma. While no genetic risk factors for Ewing sarcoma have been found, it is more common in White people, males, and teens or young adults.

No known lifestyle-related or environmental causes of gene changes lead to Ewing sarcoma.

Can Ewing sarcoma be prevented?

The risk of many adult cancers can be reduced with certain lifestyle changes (such as staying at a healthy weight or quitting smoking), but at this time there are no known ways to prevent most cancers in adolescents and young adults.

The only known risk factors for Ewing sarcoma (age, sex, and race/ethnicity) can’t be changed. There are no known lifestyle-related or environmental causes of Ewing tumors, so at this time there is no known way to protect against these cancers.