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Ewing Sarcoma

What is 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 a type of cancer that grows in bones or in the soft tissue around bones.

What is 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 symptoms

Symptoms of Ewing sarcoma depend on where the tumor is located. Symptoms might include:

  • Swelling or a lump around the tumor site
  • Fever
  • Bone pain
  • Limping or problems walking
  • Bone that breaks without a reason

Sarcomas are cancer found in the bone and soft tissue of the body. Watch this video to learn about sarcoma symptoms, diagnosis, and treatment in children.

Pain can get worse over weeks or months. It may even cause your child to wake up at night.

The signs and symptoms of Ewing sarcoma often start out like normal childhood aches and pains. This means that it can take a while before Ewing sarcoma is diagnosed.

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.

X-ray of a pediatric cancer patient's femur with markings to indicate Ewing sarcoma

X-ray of a child’s femur showing the size of the Ewing sarcoma

Ewing sarcoma diagnosis

Doctors use several procedures and tests to diagnose Ewing sarcoma. These include:

  • A health history, physical exam, and blood tests to learn about symptoms, general health, past illnesses, and risk factors. 
    • A complete blood count (CBC) 
    • A test to check the level of lactate dehydrogenase (LDH). This substance in the blood may be high in patients with Ewing sarcoma.
  • Imaging tests such as: 
    • An x-ray to check for bone cancer
    • A CT scan of the chest to see if Ewing sarcoma has spread to the lungs
    • An MRI of the primary (first) tumor to evaluate the tumor and help plan for surgery
    • Full-body imaging using bone scans or PET scans to look for cancer in other bones and other areas of the body
    • Bone marrow aspiration and biopsy to see if cancer has spread to the inside part of the bones (bone marrow)
    • A biopsy of the mass to check for cancer

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.

Metastatic Ewing sarcoma means that the cancer has spread to other places, such as the lungs, bones, or bone marrow. Approximately 25% of patients have metastatic disease at diagnosis. About half of those patients have spread of disease to lungs at diagnosis.

Ewing sarcoma treatment

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

  1. Chemotherapy

    Chemotherapy is usually the first treatment for Ewing sarcoma. These medicines kill cancer cells or stop them from growing and making new cancer cells. The chemotherapy used includes a combination of medicines to treat the cancer.

    Your child may be treated with 3–4 months of chemotherapy before having surgery, radiation or both. Your child may need more chemotherapy after surgery or radiation therapy. The typical length of treatment for Ewing sarcoma is about 8–9 months.
     

  2. Surgery

    Surgery is used to remove the primary tumor when possible. The surgeon also removes a small amount of tissue around the tumor to make sure that no cancer cells are left behind.

    In some cases, doctors add bone or tissue taken from a healthy part of the patient to replace what was removed. Sometimes they use an artificial bone or an implant (called a prosthesis).

    In limb-sparing surgery, the doctor removes the piece of bone with the tumor. The doctor then replaces the bone with a metal prosthesis or another piece of bone.

    In some cases, the patient needs amputation to completely remove the tumor.

  3. Radiation therapy

    Radiation therapy may be used to treat Ewing sarcoma along with chemotherapy and surgery.

    Radiation is used to kill or slow the growth of cancer cells left behind after surgery or chemotherapy.

    Your child’s doctor will let you know if radiation therapy is needed.

  4. Other therapies

    Targeted therapies are newer drugs that find and attack certain types of cancer cells to stop them from growing.

    Doctors may use high-dose chemotherapy with stem cell rescue for disease that has spread or returned after treatment.

    Many children are offered treatment for Ewing sarcoma as part of a clinical trial.

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.

Micah holding his rock climbing medal

Finding a New Passion

Limb-sparing surgery saved Micah Winkle’s leg, but the Ewing sarcoma patient had to give up contact sports.

Learn how rock climbing became his passion.

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:

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?
     

Key points about Ewing sarcoma

  • Ewing sarcoma is a type of cancer that grows in bones or in the soft tissue around bones.
  • Ewing sarcoma is the second most common type of pediatric bone tumor.
  • It is usually treated with chemotherapy and surgery, sometimes with radiation. Other treatment options might include clinical trials.
  • Ewing sarcoma can recur years into survivorship.
  • Your care team will monitor your child for recurrence and help you manage late effects of treatment.

—
Reviewed: July 2023

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