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Ewing Sarcoma Treatment (PDQ®)

  • Last Modified: 08/21/2014

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General Information About Ewing Sarcoma



Ewing sarcoma is a type of tumor that forms in bone or soft tissue.

Ewing sarcoma is a type of tumor that forms from a certain kind of cell in bone or soft tissue. Other names for Ewing sarcoma are:

All of these names may be grouped together and called Ewing sarcoma family of tumors.

Ewing sarcoma may be found in the bones of the legs, arms, feet, hands, chest, pelvis, spine, or skull. Ewing sarcoma also may be found in the soft tissue of the trunk, arms, legs, head and neck, abdominal cavity, or other areas.

Ewing tumors often occur in teenagers and young adults.

Signs and symptoms of Ewing sarcoma include swelling and pain near the tumor.

These and other signs and symptoms may be caused by Ewing sarcoma or by other conditions. Check with your child’s doctor if you see any of the following in your child:

  • Pain and/or swelling, usually in the arms, legs, chest, back, or pelvis (area between the hips).
  • A lump (which may feel soft and warm) in the arms, legs, chest, or pelvis.
  • Fever for no known reason.
  • A bone that breaks for no known reason.

Tests that examine the bone and soft tissue are used to diagnose and stage Ewing sarcoma.

The following tests and procedures may be used to diagnose or stage Ewing sarcoma:

  • Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.

  • MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body, such as the area where the tumor formed. This procedure is also called nuclear magnetic resonance imaging (NMRI).

  • CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the area where the tumor formed, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.

  • PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. A PET scan and a CT scan are often done at the same time. If there is any cancer, this increases the chance that it will be found.

  • Bone scan : A procedure to check if there are rapidly dividing cells, such as cancer cells, in the bone. A very small amount of radioactive material is injected into a vein and travels through the bloodstream. The radioactive material collects in the bones and is detected by a scanner.

  • Bone marrow aspiration and biopsy : The removal of bone marrow, blood, and a small piece of bone by inserting a hollow needle into the hipbone. Samples are removed from both hipbones. A pathologist views the bone marrow, blood, and bone under a microscope to see if the cancer has spread.

  • X-ray: An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body, such as the chest or the area where the tumor formed.

  • Complete blood count (CBC): A procedure in which a sample of blood is drawn and checked for the following:

  • Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances, such as lactate dehydrogenase (LDH), released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it.

A biopsy is done to diagnose Ewing sarcoma.

Tissue samples are removed during an incisional or needle biopsy so they can be viewed under a microscope by a pathologist to check for signs of cancer. It is helpful if the biopsy is done at the same center where treatment will be given.

  • Needle biopsy: For a needle biopsy, tissue is removed using a needle. This type of needle biopsy may be done if it’s possible to remove tissue samples large enough to be used for testing.

  • Incisional biopsy: For an incisional biopsy, a sample of tissue is removed through an incision in the skin.

  • Excisional biopsy : The removal of an entire lump or area of tissue that doesn’t look normal.

For an incisional or excisional biopsy, the specialists (pathologist, radiation oncologist, and surgeon) who will treat the patient usually work together to decide where the incision should be made. This is done so that the biopsy incision doesn't affect later treatment such as surgery to remove the tumor or radiation therapy.

If there is a chance that the cancer has spread to nearby lymph nodes, one or more lymph nodes may be removed and checked for signs of cancer.

The following tests may be done on the tissue that is removed:

  • Cytogenetic analysis : A laboratory test in which cells in a sample of tissue are viewed under a microscope to look for certain changes in the chromosomes.

  • Immunohistochemistry : A test that uses antibodies to check for certain antigens in a sample of tissue. The antibody is usually linked to a radioactive substance or a dye that causes the tissue to light up under a microscope. This type of test may be used to tell the difference between different types of cancer.

  • Flow cytometry : A laboratory test that measures the number of cells in a sample, the percentage of live cells in a sample, and certain characteristics of cells, such as size, shape, and the presence of tumor markers on the cell surface. The cells are stained with a light-sensitive dye, placed in a fluid, and passed in a stream before a laser or other type of light. The measurements are based on how the light-sensitive dye reacts to the light.

Certain factors affect prognosis (chance of recovery) and treatment options.

The prognosis (chance of recovery) depends on certain factors before and after treatment.

Before treatment, prognosis depends on:

  • Whether the tumor has spread to distant parts of the body.
  • Whether the tumor has spread to nearby lymph nodes.
  • Where in the body the tumor started.
  • How large the tumor is at diagnosis.
  • Whether the tumor has certain gene changes.
  • Whether the child is younger than 15 years.
  • The patient's gender.
  • Whether the child has had treatment for a different cancer before Ewing Sarcoma.
  • Whether the tumor has just been diagnosed or has recurred (come back).

After treatment, prognosis is affected by:

  • Whether the tumor was completely removed by surgery.
  • Whether the tumor responds to chemotherapy or radiation therapy.
  • Whether the cancer came back more than two years after the initial treatment.

Treatment options depend on the following:

  • Where the tumor is found in the body and how large the tumor is.
  • Whether the tumor can be completely removed by surgery.
  • The patient's age and general health.
  • The effect the treatment will have on the patient's appearance and important body functions.
  • Whether the cancer has just been diagnosed or has recurred (come back).

Decisions about surgery may depend on how well the initial treatment with chemotherapy or radiation therapy works.