University of California San Francisco

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 scannerrotates 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 orradiation 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.

Stages of Ewing Sarcoma

The results of diagnostic and staging tests are used to find out if cancer cells have spread.

The process used to find out if cancer has spread from where it began to other parts of the body is called staging. There is no standard staging system for Ewing sarcoma. The results of the tests and procedures done to diagnose Ewing sarcoma are used to group the tumors into localized ormetastatic.

Ewing sarcoma is described based on whether the cancer has spread from the bone or soft tissue in which the cancer began.

Ewing sarcoma is described as either localized or metastatic.

Localized Ewing sarcoma

The cancer is found in the bone or soft tissue in which it began and may have spread to nearby tissue, including nearby lymph nodes.

Metastatic Ewing sarcoma

The cancer has spread from the bone or soft tissue in which it began to other parts of the body. In Ewing tumor of bone, the cancer most often spreads to the lung, other bones, and bone marrow.

There are three ways that cancer spreads in the body.

Cancer can spread through tissue, the lymph system, and the blood:

  • Tissue. The cancer spreads from where it began by growing into nearby areas.
  • Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body.
  • Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body.

Cancer may spread from where it began to other parts of the body.

When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood.

  • Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body.
  • Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body.

The metastatic tumor is the same type of cancer as the primary tumor. For example, if Ewing sarcoma spreads to the lung, the cancer cells in the lung are actually Ewing sarcoma cells. The disease is metastatic Ewing sarcoma, not lung cancer.

Recurrent Ewing Sarcoma

Recurrent Ewing sarcoma is cancer that has recurred (come back) after it has been treated. The cancer may come back in the tissues where it first started or in another part of the body.

Treatment Option Overview

There are different types of treatment for children with Ewing sarcoma.

Different types of treatments are available for children with Ewing sarcoma. Some treatments arestandard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment.

Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment.

Children with Ewing sarcoma should have their treatment planned by a team of health care providers who are experts in treating cancer in children.

Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other health care providers who are experts in treating children with Ewing sarcoma and who specialize in certain areas of medicine. These may include the following specialists:

Some cancer treatments cause side effects months or years after treatment has ended.

Side effects from cancer treatment that begin during or after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include the following:

Some late effects may be treated or controlled. It is important to talk with your child's doctors about the effects cancer treatment can have on your child. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information.)

Three types of standard treatment are used:

Chemotherapy

Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into avein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is treatment using more than one anticancer drug.

The type of chemotherapy given depends on whether the cancer is found only in the place it first formed, has spread to other parts of the body, or has come back after treatment.

Chemotherapy is part of the treatment for all patients with Ewing tumors. It is usually given to kill any tumor cells that have spread to other parts of the body. Chemotherapy may also be given to shrink the tumor before surgery or radiation therapy.

Radiation therapy

Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy. External radiation therapy uses a machine outside the body to send radiation toward the cancer. Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer.

External radiation therapy is used to treat Ewing sarcoma.

Radiation therapy is used when the tumor cannot be removed by surgery or when surgery to remove the tumor will affect the way the child will look or important body functions. It is used to make the tumor smaller and decrease the amount of tissue that needs to be removed during surgery. It may also be used to treat tumors that have spread to other parts of the body.

Surgery

Surgery is usually done to remove cancer that is left after chemotherapy or radiation therapy. When possible, the whole tumor is removed by surgery. Tissue and bone that are removed may be replaced with a graft, which uses tissue and bone taken from another part of the patient's body or a donor. Sometimes an implant, such as artificial bone, is used.

Even if the doctor removes all of the cancer that can be seen at the time of the operation, chemotherapy or radiation therapy may be given after surgery to kill any cancer cells that are left. Chemotherapy or radiation therapy given after surgery to lower the risk that the cancer will come back is called adjuvant therapy.

New types of treatment are being tested in clinical trials.

This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI Web site.

Chemotherapy with stem cell transplant

This treatment is a way of giving high doses of chemotherapy to kill cancer cells and then replacingblood -forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the bone marrow or blood of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through aninfusion. These reinfused stem cells grow into (and restore) the body’s blood cells.

Targeted therapy

Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells.

Monoclonal antibody therapy is a type of targeted therapy being studied in the treatment of recurrentEwing sarcoma. Monoclonal antibodies are made in the laboratory, from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells.

Kinase inhibitor therapy is another type of targeted therapy being studied in the treatment of recurrentEwing sarcoma. Kinase inhibitors are drugs that block a protein needed for cancer cells to divide.

Patients may want to think about taking part in a clinical trial.

For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.

Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.

Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.

Patients can enter clinical trials before, during, or after starting their cancer treatment.

Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.

Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.

Follow-up tests may be needed.

Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. This is sometimes called re-staging.

Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.

Treatment Options for Ewing Sarcoma

Localized Ewing Sarcoma

The treatment of localized Ewing sarcoma always includes chemotherapy. Surgery, radiation therapy, or both may also be used. The treatments and the order they are given in depends on the following:

  • Where in the body the tumor started.
  • The size of the tumor.
  • Whether the treatment will affect the way the child will look or important body functions.

See the Treatment Option Overview section of this summary for more information about surgery, chemotherapy, and radiation therapy used to treat children with Ewing sarcoma. New ways of givingcombination chemotherapy are being studied for localized Ewing sarcoma.

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients withlocalized Ewing sarcoma/peripheral primitive neuroectodermal tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI Web site.

Metastatic Ewing Sarcoma

The treatment of metastatic Ewing sarcoma always includes chemotherapy. Surgery, radiation therapy, or both may also be used. The treatments and the order they are given depends on the following:

  • Where in the body the tumor started.
  • Where the tumor has spread.
  • The size of the tumor.
  • Whether the treatment will affect the way the child will look or important body functions.

See the Treatment Option Overview section of this summary for more information about surgery, chemotherapy, and radiation therapy used to treat children with Ewing sarcoma.

Treatments being studied for metastatic Ewing sarcoma include the following:

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients withmetastatic Ewing sarcoma/peripheral primitive neuroectodermal tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI Web site.

Recurrent Ewing Sarcoma

Treatment of recurrent Ewing sarcoma may include the following: