Ewing's Sarcoma

Written by April Kahn | Published on August 20, 2012
Medically Reviewed by George Krucik, MD

What is Ewing’s Sarcoma?

Ewing’s sarcoma is a cancerous tumor. It usually begins in or near a bone. The tumors can form on any bone, however, they generally grow on long bones. Common locations include the bones of the upper arm and leg. Ewing’s sarcoma is considered a primary bone cancer since it develops from within the bone. However, these tumors can spread to other parts of the body, including the lungs and bone marrow.

Ewing’s sarcoma is a rare disease. An estimated 250 cases are diagnosed per year in the United States, according to The University of Texas MD Anderson Cancer Center (MDAnderson). The American Academy of Orthopedic Surgeons (AAOS) states that Ewing’s sarcoma is the second most common bone tumor in children and young adults (AAOS).

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Ewing Family of Tumors

There are four different types of Ewing’s tumors. Together they are referred to as the Ewing family of tumors (EFT). Three types occur in the bone. This group includes:

  • classic Ewing’s sarcoma of the bone
  • primitive neuroectodermal tumors (PNET)
  • Askin tumors (PNET of the chest)

Ewing’s sarcoma can also occur in types of tissue. These are known as extraosseous Ewing sarcoma or Ewing sarcoma of the soft tissue.

What Causes Ewing’s Sarcoma

Experts are not certain what causes Ewing’s sarcoma. No risk factors have been identified. There are no known ways to prevent Ewing’s sarcoma.

Scientists have found one interesting thing about patients with Ewing’s sarcoma. They have genetic mutations in chromosomes 11 and 22. However, these mutations are not inherited. Doctors are not certain when they occur.

Symptoms of Ewing’s Sarcoma

Symptoms of Ewing’s sarcoma include:

  • swelling in the area of the tumor
  • pain in the affected area

The pain may be more noticeable after an injury to the affected area. Injuries are not known to cause Ewing’s sarcoma. However, a bone weakened by a tumor may be more likely to break during an injury.

Testing for Ewing’s Sarcoma

An imaging test, such as an X-ray, is sometimes enough to diagnose Ewing’s sarcoma. A biopsy can be used for confirmation. This procedure involves taking a tissue sample from the tumor. Ewing’s sarcoma cells are easily identified under a microscope.

Other tests that are used to check for Ewing’s sarcoma are:

  • blood test
  • bone scan
  • bone marrow biopsy
  • CT scan of the lungs

Treating Ewing’s Sarcoma

If the tumor is confined to its original location, chemotherapy may be used to shrink the tumor. Chemotherapy uses medication to kill cancer cells and shrink a tumor. This procedure is followed by surgery to remove the tumor once it’s small enough. After the tumor is removed, the surgeon will reconstruct any missing bone. This can be done either with bone grafts or man-made materials.

Sometimes radiation therapy is used to treat Ewing’s sarcoma. This is more likely if the cancer has spread or if surgery is too risky. It is also used to reduce the chance of primary bone cancer returning.

Future Outlook for Patients With Ewing’s Sarcoma

The outlook of Ewing’s sarcoma differs from patient to patient. Statistics from the AAOS state that two-thirds of patients survive at least five years after a diagnosis, if the disease had not spread beyond the primary tumor (AAOS).

If you are diagnosed with Ewing’s sarcoma, you will continue to follow up with your doctor even once treatment is complete. Sometimes tumors come back. Continuous monitoring helps ensure that they are detected and treated early.

Complications of Ewing’s Sarcoma

The main complication of Ewing sarcoma is the return of the tumor. The risk of the tumor coming back is highest in the first few years after treatment.

Other complications may arise from having radiation treatment. These complications include:

  • skin damage
  • slow wound healing
  • organ damage (near the area of treatment)
  • leg or arm swelling
  • muscle scarring
  • loss of flexibility in the joints
  • development of secondary cancers caused by radiation
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