Ewing Sarcoma - Childhood and Adolescence: Risk Factors

Approved by the Cancer.Net Editorial Board, 11/2022

ON THIS PAGE: You will find out more about the factors that increase the chance of developing Ewing sarcoma. Use the menu to see other pages.

What are the risk factors for Ewing sarcoma?

A risk factor is anything that increases a person’s chance of developing cancer. Although risk factors often influence the development of cancer, most do not directly cause cancer. Some people with several risk factors never develop cancer, while others with no known risk factors do.

Doctors and researchers do not know what causes most cancers in children and young adults. But the following factors may raise a person’s chance of developing Ewing sarcoma:

  • Genetic changes. Changes in a tumor cell's chromosomes appear to be responsible for Ewing sarcoma, but the disease is not inherited. This means that it isn’t passed down from a parent to a child. The genetic changes occur for no known reason.

    A high percentage of Ewing sarcoma cells have a chromosomal translocation, which means that small pieces of genetic material have swapped places inside the tumor cell. The translocation is usually between chromosomes 11 and 22, although it may also occur between chromosomes 21 and 22, 7 and 22, and 17 and 22. The fusion of these bits of genetic material results in the out-of-control growth of Ewing sarcoma cells.

  • Age. Ewing sarcoma can occur at any age. But more than half (50%) of people with Ewing sarcoma are between the ages of 10 and 20, with a median age of 15.

  • Sex. Ewing sarcoma is more common in boys than girls.

  • Race/ethnicity. Ewing sarcoma occurs most frequently in White people and is rare in Black people in the United States and Africa. Ewing sarcoma has been reported in Japan and is uncommon in China. Recent research suggests that there are 3 susceptibility loci, which are regions of a chromosome that are inherited with an illness, for Ewing sarcoma (1p36.22, 10q21, and 15q15). A polymorphism has been identified in the EGFR2 gene, which may cooperate with and magnify the enhanced activity of the EWSR1-FLI1 fusion gene product. The polymorphism associated with the increased risk for Ewing sarcoma is found at a higher frequency in White people than in Black people or Asian people, which could explain the difference in frequency of the disease in these populations.

The next section in this guide is Symptoms and Signs. It explains what changes or medical problems Ewing sarcoma can cause. Use the menu to choose a different section to read in this guide.