Originally published by Harvard Health.

What Is It?

Ewing’s sarcoma is a very rare cancer that appears as a solid tumor, most often in children and teens. Most cases occur between ages 10 and 20, and about one quarter occur before age 10. Only a small percentage of people with Ewing’s sarcoma are older than 20. Nearly everyone with this cancer is white.

Because Ewing’s sarcoma typically develops in bones, it used to be considered a bone cancer. But it actually arises from a type of primitive nerve cell. That’s why Ewing’s sarcoma can occur outside bones, in the body’s soft tissues. When it does, it is called non-osseous Ewing’s sarcoma.

Ewing’s sarcoma usually develops in the arms or legs. However, it can occur in the pelvis, ribs, spine, and, in rare cases, other bones or soft tissues. When diagnosed, about a quarter of Ewing’s sarcomas have spread (metastasized) to another part of the body. The most common sites for a metastasis are

• the lungs
• another bone
• the bone marrow.

Like other cancers, Ewing’s sarcoma is caused by abnormal cells that multiply out of control. The cells of Ewing’s sarcoma have a change in the chemical material (DNA) that the person inherited from his or her parents. This change, called a translocation, usually involves a rearrangement of DNA between two chromosomes. The problem, however, is not inherited. It develops after birth.

Ewing’s sarcoma does not seem to be related to

• exposure to radiation
• exposure to chemicals
• other environmental factors.

No one knows what causes the genetic change that leads to Ewing’s sarcoma.

Symptoms

Symptoms of Ewing’s sarcoma include

• persistent pain and swelling in an arm or leg, which may occur at rest and may even awaken the person from sleep
• a firm lump on an arm or leg, sometimes with tenderness
• a limp (if the tumor affects the leg)
• difficulty breathing (if the tumor affects the ribs)
• fever
• weight loss.

In physically active children, the pain and swelling of Ewing’s sarcoma can be mistaken for symptoms of a sports injury. In those who have a fever, symptoms of Ewing’s sarcoma can be confused with those of a bone infection. As a result, diagnosing Ewing’s sarcoma may take time.

Diagnosis

After reviewing your symptoms, your doctor will examine you and the painful area. Your doctor will check the area for warmth, redness, swelling, tenderness, limited motion, and any signs that a joint is involved. Blood and urine tests and an x-ray of the area usually follow the physical exam.

In most cases, the results of laboratory tests are normal because blood and urine tests can’t confirm the diagnosis of Ewing’s sarcoma. However, the x-ray usually will show an abnormality consistent with Ewing’s sarcoma or another problem, such as bone destruction.

If your doctor finds x-ray evidence of a bone tumor, he or she will refer you to a hospital that has the facilities, personnel, and experience to treat bone cancer. There you will have further tests, such as magnetic resonance imaging (MRI) scans, to see whether tumor has invaded other structures.

After the tumor’s location has been determined, you will have a biopsy to confirm the diagnosis of Ewing’s sarcoma. During a biopsy, a small amount of tissue is removed and examined in a laboratory.

You probably will have a chest x-ray, CT scan, and a radionuclide bone scan. A PET scan may be ordered as well. Along with a bone-marrow biopsy, these tests can show whether the cancer has spread to your lungs, other bones, or bone marrow.

Expected Duration

Ewing’s sarcoma will continue to grow until it is treated. If it is not treated, this cancer can spread to the lungs and to other bones.

Prevention

There is no way to prevent Ewing’s sarcoma.

Treatment

Ewing’s sarcoma is treated with surgery, radiation therapy, and/or chemotherapy. The choice of treatment depends on the size of the tumor and on how much of the nearby tissue it had invaded.

To stop the tumor from growing or spreading, doctors can remove it surgically or treat it with radiation. Usually, surgeons can remove the tumor without amputating the limb. Then the surgeon fills in the area with a bone graft or artificial material (prosthesis) so that the patient can keep as much function as possible. If the cancer has spread to the lungs, surgeons open the chest to remove as much of the cancer as possible. Radiation therapy is also very effective in treating Ewing’s sarcoma.

Almost always, chemotherapy is used in addition to radiation and/or surgery. Sometimes doctors give chemotherapy before surgery, which makes it easier to remove the tumor. Chemotherapy also lowers the chance that the tumor will spread to other parts of the body.

If treatment is successful, you will need follow-up visits with your doctor for many years. This is because Ewing’s sarcoma can return in the 10 years following the diagnosis. Also, if you received radiation therapy, a second cancer may develop where you received the radiation. You also may suffer late side effects from chemotherapy. Your doctors will periodically order:

• blood tests to monitor blood counts and kidney function
• an echocardiogram to track heart function.

When to Call a Professional

Call your doctor if you or your child develops persistent or unexplained pain and swelling in a bone, with or without a fever.

Prognosis

After proper treatment, about half of those patients whose cancer has not spread survive at least five years. The prognosis is better if the tumor is located below the elbow or mid-calf. People whose cancer has spread usually have a poorer prognosis; only about a quarter of them live at least five years.

Additional Information

National Cancer Institute (NCI)
U.S. National Institutes of Health
Public Inquiries Office
Building 31, Room 10A0
331 Center Drive, MSC 8322
Bethesda, MD 20892-2580
Phone: 301-435-3848
Toll-Free: 800-422-6237
TTY: 800-332-8615
www.nci.nih.gov/

American Cancer Society (ACS)
1599 Clifton Road, NE
Atlanta, GA 30329-4251
Toll-Free: 800-227-2345
www.cancer.org/

American Academy of Pediatrics (AAP)
141 Northwest Point Blvd.
Elk Grove Village, IL 60007-1098
Phone: 847-434-4000
www.aap.org/

American Society of Clinical Oncology
1900 Duke St. Suite 200
Alexandria, VA 22314
Phone: 703-299-0150
Fax: 703-299-1044
www.asco.org/

American Academy of Orthopaedic Surgeons (AAOS)
6300 North River Road
Rosemont, IL 60018-4262
Phone: 847-823-7186
Toll-Free: 800-346-2267
orthoinfo.aaos.org/