Children - Ewing Sarcoma

What is Ewing sarcoma?

Ewing sarcoma is a rare type of cancer. It’s most common in children and teens between the ages 10 and 19. It usually grows in bone, but it can also grow in soft tissue that’s connected to the bone. This may include tendons, ligaments, cartilage, or muscles.

Ewing sarcoma most often grows in:

  • Bones of the legs, hips and pelvis, chest, and arms
  • Soft tissue of the torso, arms, and legs

What causes Ewing sarcoma in children?

The exact cause of Ewing sarcoma is not known. The cancer may be caused by changes in the DNA of the cells. These changes are not passed on from parents to children. They happen by chance.

What are the symptoms of Ewing sarcoma in children?

Symptoms can occur a bit differently in each child. They can include:

  • Pain around the site of the tumor that may come and go, but gets worse over time, with activity, and at night
  • Swelling  around the site of the tumor
  • A lump (mass)
  • Fever
  • Weight loss
  • Feeling tired

The symptoms of Ewing sarcoma can be like other health conditions. Make sure your child sees a healthcare provider for a diagnosis.

How is Ewing sarcoma in children diagnosed?

Your child's healthcare provider will ask about your child's health history and symptoms. He or she will examine your child.  Your child may be referred to a specialist. This may be a bone specialist (orthopedic surgeon) or a bone cancer specialist (orthopedic oncologist). Your child may have tests such as:

  • X-ray. An X-ray uses a small amount of radiation to take pictures of bones and other body tissues.
  • Blood tests. The blood is tested to look for signs of Ewing sarcoma. Genetic testing may also be done to look for DNA changes that can cause the cancer.
  • CT scan. This test uses a series of X-rays and a computer  to make detailed images of the body.
  • MRI. This test uses large magnets, radio waves, and a computer to make detailed images of the inside of the body.
  • Bone scan. A small amount of dye is injected into a vein. The whole body is scanned. The dye shows up in areas where there may be cancer.
  • Positron emission tomography (PET) scan. For this test, a radioactive sugar is injected into the bloodstream. Cancer cells use more sugar than normal cells, so the sugar will collect in cancer cells. A special camera is used to see where the radioactive sugar is in the body. A PET scan can sometimes spot cancer cells in different areas of the body, even when they can’t be seen by other tests. This test is often used in combination with a CT scan. This is called a PET/CT scan.
  • Bone marrow aspiration or biopsy. Bone marrow is found in the center of some bones. It’s where blood cells are made. A small amount of bone marrow fluid may be taken. This is called aspiration. Or solid bone marrow tissue may be taken. This is called a core biopsy. Bone marrow is usually taken from the hip bone. This test may be done to see if cancer cells have reached the bone marrow.
  • Tumor biopsy. A sample of the tumor is taken. It may be taken with a needle or by a surgical cut (incision). It is checked under a microscope for cancer cells. A biopsy is needed to diagnose neuroblastoma. 

Part of diagnosing cancer is called staging. Staging checks the size and location of the main tumor, if it has spread, and where it has spread. Ewing sarcoma may be stage 1, 2, or 3 with sub-stages. Talk with your child's oncologist about your child's stage and what it means. Staging also helps to decide the treatment.


Treatment will depend on the stage and other factors. The cancer can be treated with any of the below:

  • Surgery. Surgery may be done to try to save the arm or leg. This is known as limb-salvage or limb-sparing surgery. Or surgery may be done to remove an arm or leg (amputation). Surgery may be done to remove tumors in other parts of the body. Surgery is usually followed by chemotherapy or radiation therapy.
  • Chemotherapy. These are medicines that kill cancer cells. They are usually given before surgery. They may also be given after surgery. Your child may have a port or catheter inserted in order to receive the medicines into a vein (IV). Chemotherapy is given in cycles with periods of rest.
  • Radiation therapy. These are high-energy X-rays or other types of radiation. Radiation is used to kill cancer cells or stop them from growing. It may be used when a tumor is hard to remove with surgery.
  • High-dose chemotherapy with a stem cell transplant. Young blood cells (stem cells) are taken from the child or from someone else. This is followed by a large amount of chemotherapy medicine. This causes damage to the bone marrow. After the chemotherapy, the stem cells are replaced. This treatment may be used when there is a high risk of cancer returning.
  • Supportive care. Treatment can cause side effects. Medicines and other treatments can be used for pain, fever, infection, and nausea and vomiting.
  • Clinical trials. Ask your child's healthcare provider if there are any treatments being tested that may work well for your child. 

With any cancer, how well a child is expected to recover (prognosis) varies. Keep in mind:

  • Getting medical treatment right away is important for the best prognosis. Cancer that has spread is harder to treat.
  • Ongoing follow-up care during and after treatment is needed.
  • New treatments are being tested to improve outcome and to lessen side effects.

What are possible complications of Ewing sarcoma in children?

A child may have complications from the sarcoma or from treatment, such as:

  • Infection or bleeding from surgery
  • Loose or broken grafts or rods from limb-salvage surgery
  • Hair loss, mouth sores, nausea, vomiting, diarrhea, increased infections, easy bruising and bleeding, and feeling tired from chemotherapy
  • Burns, hair loss, nausea, diarrhea, poor bone growth, organ damage, and new cancers from radiation
  • Emotional and physical challenges from amputation
  • Heart and lung problems
  • Problems with growth and development
  • Learning problems
  • Changes in sexual development
  • Problems with the ability to have children (fertility) in the future
  • Return of the cancer
  • Growth of other cancers

How is Ewing sarcoma in children managed?

A child with a Ewing sarcoma needs ongoing care. Your child will be seen by oncologists and other healthcare providers to treat any late effects of treatment and to watch for signs or symptoms of the tumor returning. Your child will be checked with imaging tests and other tests. And your child may see other healthcare providers for problems from the tumor or from treatment. Your child may need therapy to help with movement and muscle strength. This may be done by physical and occupational therapists.

You can help your child manage his or her treatment in many ways. For example:

  • Your child may have trouble eating. A dietitian may be able to help.
  • Your child may be very tired. He or she will need to balance rest and activity. Encourage your child to get some exercise. This is good for overall health. And it may help to lessen tiredness.
  • If your child smokes, help him or her quit. If your child doesn’t smoke, make sure he or she knows the danger of smoking.
  • Get emotional support for your child. Find a counselor or child support group can help.
  • Make sure your child attends all follow-up appointments.

When should I call my child’s healthcare provider?

Call the healthcare provider if your child has:

  • Symptoms that get worse
  • New symptoms
  • Side effects from treatment

Key points about Ewing sarcoma in children

  • Ewing sarcoma is a rare group of cancers that usually occur in the bone, but can also occur in soft tissue. 
  • X-ray and other imaging tests may be used in diagnosing Ewing sarcoma. A biopsy is needed to make a definite diagnosis.
  • Ewing sarcoma is treated with chemotherapy followed by surgery and/or radiation therapy. High-dose chemotherapy and stem cell transplant may be used in some children.
  • Continuous follow-up care during and after treatment is necessary.
  • Many complications can occur from the cancer and from the treatment. The complications may be short- or long-term.

Next steps

Tips to help you get the most from a visit to your child’s health care provider:
  • Before your visit, write down questions you want answered.
  • At the visit, write down the names of new medicines, treatments, or tests, and any new instructions your provider gives you for your child.
  • If your child has a follow-up appointment, write down the date, time, and purpose for that visit.
  • Know how you can contact your child’s provider after office hours. This is important if your child becomes ill and you have questions or need advice.
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