Ewing Sarcoma

Ewing sarcoma is a type of cancer that grows in bones or in the soft tissue around bones. It often occurs in the leg, pelvis, ribs, or arm.

Ewing sarcoma is the second most common type of pediatric bone tumor. But it is rare. Only about 200 children and young adults are diagnosed with Ewing sarcoma in the United States each year.

Ewing sarcoma is more common in older children and teens.

If the cancer is caught before it spreads, the chance of long-term survival is about 70%. If the cancer has already spread, the chance of a cure is about 30%.

Ewing sarcoma is usually treated with chemotherapy, surgery and/or radiation. Patients can have treatment that has already been studied in clinical trials. Or they may get treatment that is being studied at the time of diagnosis.

Ewing sarcoma risk factors

We do not know why children and young adults develop Ewing sarcoma. But certain factors can increase the risk. These factors include:

• Age – About half of Ewing sarcoma tumors occur in children and teens 10–20 years of age.
• Sex – Ewing sarcoma occurs slightly more often in males than females.
• Race – Whites have a higher risk of developing Ewing sarcoma than Blacks and Asians do.
• Prior exposure – Cancer survivors who had radiation therapy have a higher risk of developing Ewing sarcoma.

Ewing sarcoma does not seem to be passed down in families.

Ewing sarcoma staging

Although other cancers are classified as stages 1–4, Ewing sarcoma is classified as either localized or metastatic.

Localized means there is a tumor in only 1 place in the body.

Metastatic means the cancer has spread to 1 or more places. These might include:

• Lungs
• Lymph nodes
• Bones
• Bone marrow

About 25% of patients have metastatic disease when they are diagnosed. In about half of those patients, the disease has spread to the lungs.

Ewing sarcoma is usually treated with chemotherapy followed by surgery and more chemotherapy. Radiation therapy may be used in place of surgery or added to the treatment.

Clinical trials are studying new treatments for Ewing sarcoma. These include targeted therapy and immunotherapy.

Ewing sarcoma has 3 main treatment goals:

1. Shrink the primary tumor (the first tumor) and kill any other cancer cells in the body
2. Remove the primary tumor
3. Treat any remaining cancer to reduce risk that the cancer will return

Ewing sarcoma prognosis

Survival of Ewing sarcoma depends on several factors:

• Size and location of the tumor
  • Large tumor size is linked to worse survival.
  • Cancer in the pelvis, ribs, or spine is harder to treat.
  • Patients with Ewing sarcoma tumors in soft tissue without bone involvement tend to have improved survival compared to those with a tumor in the bone.
• Patient age and gender
  • Patients under age 15 tend to have improved survival than older patients.
  • Girls tend to have a better prognosis than boys.
• Cancer that has spread to other parts of the body (metastatic)
  • Patients with metastatic tumors in the lungs do better than patients with metastatic tumors in the bones or lymph nodes.
• How the tumor responds to chemotherapy or radiation therapy
• Whether the cancer has come back

Patients who have chemotherapy and a single, localized Ewing sarcoma tumor that can be completely removed with surgery have a 70% chance of long-term cure in the United States.

Patients who have cancer that has spread to distant parts of the body at diagnosis have a 15–30% chance of survival, depending on where the cancer has spread.

The prognosis for patients with recurrent Ewing sarcoma is poor. The chance of cure after the cancer has returned (relapsed) is around 10–15% in the U.S.

Patients with recurrent Ewing sarcoma often develop new tumors within the first 2 years of diagnosis. This most often occurs as new growths in the lungs.

Patients who have early recurrence (their Ewing sarcoma comes back within 2 years of diagnosis) are much harder to cure than those who have later recurrence.

Balancing quality of life with cancer-directed therapy is important. Talk to your care team about problems to expect and how to help manage them. Palliative care can help families manage symptoms, promote quality of life, and make informed decisions.

Life after Ewing sarcoma 

Monitoring for Ewing sarcoma recurrence

Ewing sarcoma can come back years into survivorship. But it is most common within the first 2 years after treatment ends. The tumor can come back in the same place or in another part of the body.

Your child will need follow-up care to screen for recurrence for a long time after treatment ends. The care team will suggest types of tests and how often they should be done. Imaging tests usually include:

• CT scans of the lungs
• X-rays or MRI of the primary tumor site
• PET or bone scans

If your child has had Ewing sarcoma in the bone marrow, the care team may also suggest bone marrow tests.

Life after limb-sparing surgery or amputation 

Most patients who have an amputation or limb-sparing surgery do well over time.

They report good physical function and quality of life. Follow-up care is important.

Your child should have an annual exam to check muscle and bone function. It is important to make sure there are no ongoing problems. Uneven limb length, changes in the way your child walks, joint issues, or other problems can cause chronic pain or disability.

• If your child had an amputation, they should have an annual exam to keep the function of their prosthetic limb.
• If your child had a bone graft and/or metal implant, an orthopedic surgeon should see your child once a year.
• If your child had limb-sparing surgery, they may need other surgeries to lengthen the limb until they reach adult size.

Health after Ewing sarcoma

Children treated for Ewing sarcoma are at risk side effects related to therapy. Your child needs to have regular physical checkups and screenings. Living a healthy lifestyle with healthy eating habits can help, too.

Survivors of bone cancer tend to be less active. Regular exercise is important to maintain health, fitness, and physical function.

If your child had systemic chemotherapy or radiation, they need to be monitored for acute and late effects of therapy. Problems due to treatment may include:

• Hearing loss
• Heart problems
• Kidney damage
• Infertility

Radiation to the leg can affect growth in that bone. It can cause the leg to be shorter. This may result in unequal leg length. There are different ways to treat this problem, depending on the severity.

Your child’s care team should give you a survivorship care plan after treatment ends. This report will include needed screening tests and tips for a healthy lifestyle.

Talk to your child’s health care provider about late effects of treatment. Always follow the instructions from your care team.

Questions to ask your child's care team

• What are our treatment options?
• What are the possible side effects of each treatment?
• What can be done to manage side effects?
• Will my child need to be in the hospital for treatment?
• Where is the treatment available? Is it close to home or will we have to travel?
• Will my child have physical limitations after surgery/treatment?