Ewing Sarcoma Family of Tumors Treatment (PDQ®)

Ewing sarcoma family of tumors is a group of cancers of the bone and of soft tissue.

Ewing sarcoma family of tumors is a group of tumors that form from a certain kind of cell in bone or soft tissue. This family of tumors includes the following:

  • Ewing tumor of bone. This type of tumor is found in the bones of the legs, arms, chest, trunk, back, or head. There are three types of Ewing tumor of bone:
    • Classic Ewing sarcoma.
    • Primitive neuroectodermal tumor (PNET).
    • Askin tumor (PNET of the chest wall).
  • Extraosseous Ewing sarcoma (tumor growing in tissue other than bone). This type of soft tissue tumor is found in the trunk, arms, legs, head, and neck.

In some patients, the tumor may have spread by the time it is diagnosed.

Ewing tumors usually occur in teenagers and are more common in boys and Caucasians.

Possible signs of Ewing sarcoma family of tumors include swelling and pain near the tumor.

These and other symptoms may be caused by Ewing sarcoma family of tumors. Other conditions may cause the same symptoms. A doctor should be consulted if any of the following problems occur:

  • Pain and/or swelling, most commonly in the arms, legs, chest, back, or pelvis (area between the hips).
  • A lump (which may feel 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 family of tumors.

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

  • 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.
  • Complete blood count (CBC): A procedure in which a sample of blood is drawn and checked for the following:
    • The number of red blood cells, white blood cells, and platelets.
    • The amount of hemoglobin (the protein that carries oxygen) in the red blood cells.
    • The portion of the blood sample made up of red blood cells.
  • 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.
  • Sedimentation rate: A procedure in which a sample of blood is drawn and checked for the rate at which the red blood cells settle to the bottom of the test tube.
  • 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.
  • 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. 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 chest, 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.
  • 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 look for signs of cancer.
  • 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.
  • 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 biopsy is done to diagnose Ewing sarcoma family of tumors.

Cells and tissues are removed during a biopsy so they can be viewed under a microscope by a pathologist to check for signs of cancer. The specialists (pathologist, radiation oncologist, and surgeon) who will treat the patient usually work together to plan the biopsy. This is done so that the biopsy incision doesn't affect later treatment with surgery to remove the tumor and radiation therapy. It is helpful if the biopsy is done at the same center where treatment will be given.

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

  • Light and electron microscopy: A laboratory test in which cells in a sample of tissue are viewed under regular and high-powered microscopes to look for certain changes in the cells.
  • 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.
  • Reverse-transcription polymerase chain reaction test (RT-PCR): A laboratory test in which cells in a sample of tissue are studied using chemicals to look for certain changes in the genes.
  • Immunohistochemistry study: A laboratory test in which a substance such as an antibody, dye, or radioisotope is added to a sample of tissue to test for certain antigens. This type of study is used to tell the difference between different types of cancer.
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.
  • Where in the body the tumor started.
  • How large the tumor is at diagnosis.
  • Whether the tumor has certain genetic changes.
  • The patient's age. Infants and patients aged younger than 15 years have a better prognosis than adolescents aged 15 years and older, young adults, or adults.
  • The patient's gender. Girls have a better prognosis than boys.
  • 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 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.
  • 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.

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 family of tumors. The results of the tests and procedures done to diagnose Ewing sarcoma family of tumors are used to group the tumors into localized or metastatic.

Ewing sarcoma family of tumors are grouped based on whether the cancer has spread from the bone or soft tissue in which the cancer began.

Ewing sarcoma family of tumors are described as either localized or metastatic.

Localized Ewing sarcoma family of tumors

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

Metastatic Ewing sarcoma family of tumors

The cancer has spread from the bone or soft tissue in which the cancer 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.

The three ways that cancer spreads in the body are:

  • Through tissue. Cancer invades the surrounding normal tissue.
  • Through the lymph system. Cancer invades the lymph system and travels through the lymph vessels to other places in the body.
  • Through the blood. Cancer invades the veins and capillaries and travels through the blood to other places in the body.

When cancer cells break away from the primary (original) tumor and travel through the lymph or blood to other places in the body, another (secondary) tumor may form. This process is called metastasis. The secondary (metastatic) tumor is the same type of cancer as the primary tumor. For example, if bone cancer spreads to the lung, the cancer cells in the lung are actually bone cancer cells. The disease is metastatic bone cancer, not lung cancer.

Recurrent Ewing sarcoma family of tumors 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.

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

Different types of treatments are available for children with Ewing sarcoma family of tumors. Some treatments are standard (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 family of tumors 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 family of tumors and who specialize in certain areas of medicine. These may include the following specialists:

  • Surgical oncologist or orthopedic oncologist.
  • Radiation oncologist.
  • Pediatric nurse specialist.
  • Social worker.
  • Rehabilitation specialist.
  • Psychologist.
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:

  • Physical problems.
  • Changes in mood, feelings, thinking, learning, or memory.
  • Second cancers (new types of cancer). Patients treated for Ewing sarcoma family of tumors have an increased risk of developing acute myeloid leukemia, myelodysplastic syndrome, and sarcomas in the area treated with radiation therapy.

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

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 a vein 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 way the chemotherapy is given depends on the type of the cancer being treated and whether it is found at the place it first formed only or whether it has 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 entire tumor is removed by surgery. Tissue and bone that are removed may be replaced with a graft, using tissue and bone taken from another part of the patient's body or a donor, or with an implant such as artificial bone.

Radiation therapy

Radiation therapy may be used to shrink the tumor before surgery so less tissue needs to be removed. It may also be used to kill tumor cells that are left after surgery or chemotherapy. 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. The way the radiation therapy is given depends on the type of the cancer being treated and whether it is found at the place it first formed only or whether it has spread to other parts of the body.

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

Stem cell transplant is a way of replacing blood-forming cells destroyed by chemotherapy. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. 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 recurrent Ewing 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.

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 condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.

A link to a list of current clinical trials is included for each treatment section. For some types or stages of cancer, there may not be any trials listed. Check with your doctor for clinical trials that are not listed here but may be right for you.

Treatment of localized Ewing sarcoma family of tumors may include the following:

  • Combination chemotherapy followed by surgery and/or radiation therapy.
  • Surgery and/or radiation therapy, followed by chemotherapy, for tumors only in the skin or tissue just below the skin.
  • A clinical trial of new ways of giving combination chemotherapy.

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with localized 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. General information about clinical trials is available from the NCI Web site.

Treatment of metastatic Ewing sarcoma family of tumors may include the following:

  • Combination chemotherapy followed by radiation therapy to the area where the tumor first formed and the places where the tumor has spread.
  • A clinical trial of chemotherapy with either stem cell transplant or radiation therapy, for tumors that have spread to the lungs.

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with metastatic 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. General information about clinical trials is available from the NCI Web site.

Treatment of recurrent Ewing sarcoma family of tumors may include the following:

  • Surgery followed by combination chemotherapy.
  • Combination chemotherapy.
  • High-dose chemotherapy and sometimes a stem cell transplant using the patient's stem cells.
  • Radiation therapy or surgery to remove bone tumors, as palliative therapy to reduce symptoms and improve the quality of life.
  • Radiation therapy followed by surgery to remove tumors that have spread to the lungs.
  • A clinical trial of targeted therapy with a monoclonal antibody.
  • A clinical trial of new combinations of drugs.

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with recurrent 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. General information about clinical trials is available from the NCI Web site.

For more information from the National Cancer Institute about the Ewing sarcoma family of tumors, see the following:

For more childhood cancer information and other general cancer resources from the National Cancer Institute, see the following:

The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.

Changes were made to this summary to match those made to the health professional version.

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