Ewing’s sarcoma is a rare cancerous tumor of bone or soft tissue. It occurs mostly in young people.

Overall, it affects 1 out of every 1 million Americans. But for adolescents aged 10 to 19 years, this jumps to about 10 cases per 1 million Americans in this age group.

This means that about 200 cases are diagnosed in the United States each year.

The sarcoma is named for the American doctor James Ewing, who first described the tumor in 1921. It isn’t clear what causes Ewing’s, so there are no known methods of prevention. The condition is treatable, and, if caught early, full recovery is possible.

Keep reading to learn more.

The most common symptom of Ewing’s sarcoma is pain or swelling in the area of the tumor.

Some people may develop a visible lump on the surface of their skin. The affected area may also be warm to the touch.

Other symptoms include:

  • loss of appetite
  • fever
  • weight loss
  • fatigue
  • general unwell feeling (malaise)
  • a bone that breaks without a known reason
  • anemia

Tumors typically form in the arms, legs, pelvis, or chest. There may be symptoms specific to the location of the tumor. For example, you may experience shortness of breath if the tumor is located in your chest.

The exact cause of Ewing’s sarcoma isn’t clear. It isn’t inherited, but it can be related to non-inherited changes in specific genes that happen during a person’s lifetime. When chromosomes 11 and 12 exchange genetic material, it activates an overgrowth of cells. This may lead to the development of Ewing’s sarcoma.

Research to determine the particular type of cell in which Ewing’s sarcoma originates is ongoing.

Although Ewing’s sarcoma can develop at any age, more than 50 percent of people with the condition are diagnosed in adolescence. The median age of those affected is 15.

In the United States, Ewing’s sarcoma is nine times more likely to develop in Caucasians than in African-Americans. The American Cancer Society reports that the cancer rarely affects other racial groups.

Males may also be more likely to develop the condition. In a study of 1,426 people affected by Ewing’s, 59 percent were male and 41 percent were female.

If you or your child experience symptoms, see your doctor. In about 25 percent of cases, the disease has already spread, or metastasized, by the time of diagnosis. The sooner a diagnosis is made, the more effective treatment may be.

Your doctor will use a combination of the following diagnostic tests.

Imaging tests

This may include one or more of the following:

  • X-rays to image your bones and identify the presence of a tumor
  • MRI scan to image soft tissue, organs, muscles, and other structures and show details of a tumor or other abnormalities
  • CT scan to image cross-sections of bones and tissues
  • EOS imaging to show the interaction of joints and muscles while you’re standing
  • bone scan of your whole body to show if a tumor has metastasized
  • PET scan to show whether any abnormal areas seen in other scans are tumors

Biopsies

Once a tumor has been imaged, your doctor can order a biopsy to look at a piece of the tumor under a microscope for specific identification.

If the tumor is small, your surgeon may remove the whole thing as part of the biopsy. This is called an excisional biopsy, and it’s done under general anesthesia.

If the tumor is larger, your surgeon may cut away a piece of it. This may be done by cutting through your skin to remove a piece of the tumor. Or your surgeon may insert a large, hollow needle into your skin to remove a piece of the tumor. These are called incisional biopsies and are usually done under general anesthesia.

Your surgeon may also insert a needle into the bone to take out a sample of fluid and cells to see if the cancer has spread into your bone marrow.

Once the tumor tissue is removed, there are several tests that help identify an Ewing’s sarcoma. Blood tests may also contribute helpful information for treatment.

Types of Ewing’s sarcoma

Ewing’s sarcoma is categorized by whether the cancer has spread from the bone or soft tissue in which it started. There are three types:

  • Localized Ewing’s sarcoma: The cancer hasn’t spread to other areas of the body.
  • Metastatic Ewing’s sarcoma: The cancer has spread to the lungs or other places in the body.
  • Recurrent Ewing’s sarcoma: The cancer doesn’t respond to treatment or returns after a successful course of treatment. It most often recurs in the lungs.

Treatment for Ewing’s sarcoma depends on where the tumor originates, the size of the tumor, and whether the cancer has spread.

Typically, treatment involves one or more approaches, including:

Treatment options for localized Ewing’s sarcoma

The common approach for a cancer that hasn’t spread is a combination of:

  • surgery to remove the tumor
  • radiation to the tumor area to kill any remaining cancer cells
  • chemotherapy to kill possible cancer cells that have spread, or micrometastasies

Researchers in one 2004 study found that combination therapy such as this was successful. They discovered the treatment resulted in a 5-year survival rate of approximately 89 percent and an 8-year survival rate of about 82 percent.

Depending on the tumor site, further treatment may be necessary after surgery to replace or restore limb function.

Treatment options for metastasized and recurrent Ewing’s sarcoma

Treatment for Ewing’s sarcoma that has metastasized from the original site is similar to that for the localized disease, but with a much lower success rate. Researchers in one 2010 study reported that the 5-year survival rate after treatment for metastasized Ewing’s sarcoma was about 70 percent.

There is no standard treatment for recurrent Ewing’s sarcoma. Treatment options vary depending on where the cancer returned and what the previous treatment was.

Many clinical trials and research studies are ongoing to improve treatment for metastasized and recurrent Ewing’s sarcoma. These include:

  • stem cell transplants
  • immunotherapy
  • targeted therapy with monoclonal antibodies
  • new drug combinations

As new treatments develop, the outlook for people affected by Ewing’s sarcoma continues to improve. Your doctor is your best resource for information about your individual outlook and life expectancy.

The American Cancer Society reports that the 5-year survival rate for people who have localized tumors is about 70 percent.

For those with metastasized tumors, the 5-year survival rate is 15 to 30 percent. Your outlook may be more favorable if the cancer hasn’t spread to organs other than the lungs.

The 5-year survival rate for people with recurrent Ewing’s sarcoma is 10 to 15 percent.

There are many factors that may affect your individual outlook, including:

  • age when diagnosed
  • tumor size
  • tumor location
  • how well your tumor responds to chemotherapy
  • blood cholesterol levels
  • previous treatment for a different cancer
  • gender

You can expect to be monitored during and after treatment. Your doctor will periodically retest to determine whether the cancer has spread.

People who have Ewing’s sarcoma may have a higher risk of developing a second kind of cancer. The American Cancer Society notes that as more young people with Ewing’s sarcoma are surviving to adulthood, the long-term effects of their cancer treatment may become apparent. Research in this area is ongoing.