Ewing sarcoma is the second most common bone cancer in children and younger adults. However, it is still very rare.

Ewing tumors account for about 1% of all childhood cancers, affecting approximately one in a million individuals.

This article discusses how common Ewing sarcoma is, statistics, survival rates, and more.

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Ewing sarcoma is a rare bone cancer that mainly affects children, adolescents, and young adults.

It can develop throughout various locations in the body. Ewing tumors may occur in any bone or soft tissue, but the most commonly affected sites are the pelvis and the long bones of the legs and arms.

Additional statistics for Ewing sarcoma are as follows:

  • An estimated 87% of Ewing sarcomas affect the bone. These sarcomas may also affect surrounding soft tissue.
  • Ewing sarcoma accounts for 10–15% of all bone sarcomas and less than 5% of all soft tissue sarcomas.
  • One-third of all Ewing tumors are present in the pelvis and spine.

Ewing sarcomas originate in bone and soft tissue, potentially affecting structures such as muscles, nerves, tendons, vessels, and joints.

The survival rate of Ewing sarcoma depends on the stage, which is identified by how far the cancer has spread beyond the bone or other primary site. There are different survival rates for different stages.

The ACS uses a 5-year relative survival rate for people with Ewing sarcoma. This measures the likelihood that a person with Ewing sarcoma will live for at least 5 years after diagnosis, compared with the overall population.

According to data from 2010–2016, the survival rates for people diagnosed with Ewing tumors are as follows:

SEER stageStage description 5-year relative survival rate
LocalizedThere are no signs indicating the cancer has spread.82%
RegionalThe cancer has spread to nearby structures or lymph nodes.70%
DistantThe cancer reached the lungs or other distant structures. 39%
All stages combinedThe average of all of the above stages combined. 62%

For example, people with a localized Ewing tumor are 82% as likely as those without cancer to live for at least 5 years after diagnosis, on average.

Ewing sarcoma cancer can spread — a process known as metastasis — and return, also called recurrence.

The survival rate decreases when the cancer metastasizes, becoming 30% for individuals with isolated lung metastases and less than 20% when the bone and bone marrow are affected.

Along with the stage of cancer, factors that may affect survival rates include:

The survival rate of Ewing sarcoma has improved over the past 40 years due to advancements in treatment options, including local and systemic therapies such as chemotherapy and radiation therapy.

However, survival rates are estimates, and the data does not predict what will happen in every case. A person can talk with medical professionals to better understand how individual factors may affect the survival rate.

Ewing sarcoma mainly affects children, teenagers, adolescents, and young adults. Around 80% of people are under 20 years of age at diagnosis.

According to the National Library of Medicine, the median age is 15 years, with the peak age ranging between 10 to 15. Most Ewing tumors occur in teens, but they can also affect younger children and adults in their 20s and 30s.

Around 30% of Ewing tumors arise in children under 10 years, with another 30% of cases affecting adults over the age of 20. Statistics on Ewing sarcoma in older people remain unknown.

Slightly more males than females develop Ewing sarcoma. Males are about three times more likely to develop Ewing tumors than females.

White adolescents face the highest risk of developing Ewing sarcoma. White people are seven times more likely to develop it than Black people, with an intermediate incidence for Asian and Hispanic individuals.

Early detection plays a crucial part in the outlook for Ewing sarcoma. According to the American Cancer Society, survival is possible for more than 80% of people with a localized Ewing sarcoma — one that has not spread to other areas.

If the tumor is present in an extremity, such as an arm or leg, professionals may consider amputation to completely remove all traces of the cancer.

Completely removing a limb means the cancer is less likely to reoccur. Surgically removing Ewing sarcoma on the extremities may be less challenging than treating tumors affecting the chest wall or pelvis.

Therefore, people with Ewing sarcoma in the arm or leg may have a better outlook than those with an Ewing tumor affecting the trunk.

Additionally, those under 10 years of age, a smaller tumor, and typical LDH levels, may have a better outlook.

This section answers common questions about Ewing sarcoma.

What is the leading cause of Ewing sarcoma?

Experts are generally unsure of the cause of Ewing sarcoma. Experts have not found an association between Ewing sarcoma and a family history of cancer, environmental risks, or past exposure to drugs and radiation.

How many cases of Ewing’s sarcoma are there a year?

There are more than 200 cases of Ewing sarcoma diagnosed in children and teens in the United States each year.

Between 1973 and 2004, the incidence of Ewing sarcoma in the U.S. was 2.93 per one million people.

Ewing sarcoma is a rare form of bone cancer affecting children and young adults. It accounts for about 1% of all childhood cancers, affecting approximately one in a million individuals.

The survival rate has improved over the last 40 years as a result of advancements in treatment options, such as chemotherapy and radiation therapy.