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Ewing Sarcoma

Other names/related types: Ewing’s sarcoma, peripheral primitive neuroectodermal tumor, pPNET, Askin tumor

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

Ewing sarcoma often arises in the bone. However, a small percent of patients develop tumors outside the bone (extraosseus). 

Ewing sarcoma is most often seen in older children and adolescents. If the cancer is caught early before the disease has spread, the chance of long-term survival is about 70%. Chance of cure is much lower if the disease has spread.

Risk Factors and Causes of Ewing Sarcoma

It is not known why Ewing sarcoma develops in some children, but certain factors may increase risk including age, gender, and ethnicity. 

  • About half of Ewing sarcoma tumors are diagnosed in older children and teens 10-20 years of age. 
  • Ewing sarcoma occurs slightly more often in males than females.
  • Caucasians have a higher risk of Ewing sarcoma compared to African-American and Asian-American children.
  • Cancer survivors who were treated with radiation therapy have a higher risk for developing Ewing sarcoma.
  • Ewing sarcoma does not seem to be passed down in families.

Signs and Symptoms of Ewing Sarcoma

Symptoms of Ewing sarcoma depend on where the tumor is located. Common signs and symptoms of Ewing sarcoma include:

  • Swelling, lump, and/or pain around the tumor site
  • Fever
  • Bone pain, especially during physical activity or at night
  • Limping or problems walking 
  • Bone that breaks without a reason

Pain may worsen over weeks or months and may cause the child to wake up at night. Because the symptoms of Ewing sarcoma can be mistaken for normal pains or injuries of childhood, it can take a while before a diagnosis is made.

Diagnosis of Ewing Sarcoma

Several types of procedures and tests are used 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) will test the number of red blood cells, white blood cells, and platelets in the blood. 
    • Doctors will also check the level of lactate dehydrogenase (LDH), a substance in the blood that may be elevated in Ewing sarcoma.
  • Imaging tests to look for tumors and help determine other tests that are needed. 
    • An X-ray of the bone.
    • A CT scan of the chest is used to see if Ewing sarcoma has spread to the lungs.
    • An MRI is used to assess the tumor and assist in planning surgery.
    • Full body imaging using bone scans or PET scans is used to look for cancer in bone and other areas of the body.
  • Bone marrow aspiration and biopsy to see if cancer has spread. Doctors will obtain bone marrow sample by inserting a thin, hollow needle into the hipbone. A pathologist will view the bone marrow under a microscope to look for signs of cancer.
  • A biopsy of the tumor to make a correct diagnosis. In a biopsy, the doctor removes a small amount of tissue from the tumor. The cells are then examined with a microscope to look for signs of cancer. An incisional biopsy or core needle biopsy may be used depending on tumor location. Ewing sarcoma tumor cells appear as small, round, blue cells under a microscope.
X-ray of a pediatric cancer patient's femur with markings to indicate Ewing sarcoma

X-ray of a pediatric cancer patient's femur marking the size of a Ewing sarcoma

X-ray shows Ewing sarcoma on the hip of a pediatric cancer patient

X-ray shows Ewing sarcoma on the hip of a pediatric cancer patient

Chest CT of a Ewing sarcoma patient with markings to show metastasis

CT scan of the chest shows that Ewing sarcoma spread to the lungs

Staging of Ewing Sarcoma

Ewing sarcoma is usually described as either localized or metastatic. Localized disease means that there is a tumor in only one place in the body. 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.

Prognosis for Ewing Sarcoma

The chance of recovery from Ewing sarcoma depends on several factors:

  • Size and location of the tumor
    • Large tumor size is associated with worse outcome. 
    • Cancer located in the pelvis, ribs, or spine is harder to treat.
    • Patients with Ewing sarcoma tumors in soft tissue (extraosseus) tend to do better compared to patients with tumor arising in the bone.
  • Patient age and gender
    • Younger patients (under age 15) tend to do better compared to older patients.
    • Girls tend to have a better prognosis compared to boys.
  • Whether the cancer has spread (metastasized) to or other parts of the body (metastatic disease). Patients with metastatic tumors in the lungs do better compared to patients with metastatic tumors in the bones or lymph nodes.
  • How responsive the tumor is to chemotherapy or radiation therapy
  • If the cancer is recurrent (has come back)

Patients with a single, localized Ewing sarcoma tumor that can be completely removed with surgery have 70% chance of long term cure. Patients who have metastatic disease in which the cancer has spread to distant parts of the body at diagnosis, (metastatic Ewing sarcoma), the chance of survival is 15-30%, depending on the location of the metastatic disease. The prognosis for patients with recurrent Ewing sarcoma is poor, and chance of cure is approximately 10%-15%.

Patients with recurrent Ewing sarcoma often develop new tumors within the first 2 years from diagnosis. This most often occurs as new nodules in the lungs. Patients who have “early recurrence” within 2 years from diagnosis are much more difficult to cure than those who have “later recurrence”.

Treatment of Ewing Sarcoma

When Ewing sarcoma is diagnosed, it is usually first treated with chemotherapy followed by surgery and/or radiation therapy. New treatments including targeted therapy and immunotherapy are also being studied in clinical trials. 

There are 3 main goals of treatment for Ewing sarcoma:

  1. Shrink the primary tumor and kill any circulating cancer cells with systemic chemotherapy
  2. Remove the primary tumor with surgery and/or radiation
  3. Treat any remaining cancer with chemotherapy to reduce risk for recurrence
  1. Chemotherapy (“chemo”) is usually the first treatment for Ewing sarcoma. These medicines kill cancer cells or stop them from growing and making new cancer cells. Often, more than one type of medicine will be used to treat the cancer. Most chemotherapy is given through injections into a central IV, but some chemo may be given by mouth. Chemotherapy is usually given before surgery/radiation to make the tumor smaller. Additionally, chemotherapy is often given after surgery/radiation to remove any remaining cancer cells and to help keep the tumor from coming back.

    Prior to surgery, patients are treated with approximately 3-4 months of chemotherapy. The regimen often includes alternating cycles of chemotherapy including: vincristine, doxorubicin, cyclophosphamide with ifosfamide, and etoposide. The chemotherapy treatments are given approximately every 2 weeks. Additional chemotherapy is given after surgery and/or radiation therapy. A typical length of treatment for Ewing sarcoma is approximately 8-9 months.

  2. Surgery is used to remove the cancer when possible. A small amount of tissue surounding the tumor is also removed to make sure that no cancer is left behind.

    In some cases, doctors will add on bone or tissue from the patient to replace what was removed. An artificial bone, or implant, may also be used. In limb-sparing surgery, the doctor removes the piece of bone with the tumor and replaces the bone with a metal prosthesis or another piece of bone. However, in some cases, amputation may be needed to make sure that the tumor is completely removed.

  3. Radiation therapy may be used to treat Ewing sarcoma in combination with chemotherapy and surgery. Radiation may be given to try to kill or slow the growth of cancer cells left behind after surgery or chemotherapy.

  4. Targeted therapies are newer drugs that act on specific targets of cancer cells to stop them from growing. Kinase inhibitors and monoclonal antibodies are being studied in the treatment of Ewing sarcoma. Kinase inhibitors act to block a protein that signals cancer cells to grow. Monoclonal antibody therapy uses special proteins that attach to cancer cells to kill the cells or slow cell growth. 

    High-dose chemotherapy with stem cell rescue may be tried as a treatment for metastatic or recurrent disease. 

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

Life after Ewing Sarcoma

Monitoring for Recurrence

Ewing sarcoma can recur years into survivorship. However, recurrence in Ewing sarcoma is most common (80%) within the first 2 years after treatment ends. The tumor can come back in the same place (local recurrence) or in another part of the body (distant recurrence).

Patients will receive follow-up care to screen for recurrence for a long time after treatment ends. The medical team will make specific recommendations for frequency and types of tests needed. Imaging tests usually include CT scans of the lungs, X-rays or MRI of the tumor site and PET or bone scans. If the patient has a history of Ewing sarcoma in the bone marrow, evaluation for recurrence may also involve testing the bone marrow.

Life after Limb-Sparing Surgery or Amputation 

Overall, most patients who undergo amputation or limb-sparing surgery do well over time. They report good physical function and quality of life. Follow-up care is important to ensure continued mobility. Patients should have an annual exam to check musculoskeletal function. It is important to make sure that there are no ongoing problems. Uneven limb length, changes in gait, joint issues, or other problems can cause chronic pain or disability. 

Patients who have undergone amputation should also have an annual exam to maintain the function of their prosthetic limbs. An orthopedic surgeon should evaluate patients with an endoprosthesis (bone graft and/or metal implant) at least once a year. Patients who have had limb-sparing surgery may require additional surgeries for limb lengthening until they reach adult height. 

Health after Cancer

Children treated for Ewing sarcoma are at risk side effects related to therapy. All survivors should continue to have regular physical checkups and screenings by a primary physician. For general health and disease prevention, survivors should adopt healthy lifestyle and eating habits. 

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

Survivors treated with systemic chemotherapy or radiation should be monitored for acute and late effects of therapy. Potential problems due to treatment may include hearing loss, heart problems, and kidney damage

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

Serious chronic health conditions are present 25 years after diagnosis in approximately 25% of survivors, according to the Childhood Cancer Survivor Study. These conditions include second cancers (increased risk following exposure to radiation), congestive heart failure, infertility or complications during pregnancy, and end-stage renal disease or renal failure.


Reviewed: June 2018