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Ewing Sarcoma Treatment (PDQ®): Treatment - Patient Information [NCI]

This information is produced and provided by the National Cancer Institute (NCI). The information in this topic may have changed since it was written. For the most current information, contact the National Cancer Institute via the Internet web site at http://cancer.gov or call 1-800-4-CANCER.

Ewing Sarcoma Treatment

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, 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.

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

These and other symptoms may be caused by Ewing sarcoma. Other conditions may cause the same symptoms. Check with your child's doctor if you see any of these problems in your child:

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:

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.

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:

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:

After treatment, prognosis is affected by:

Treatment options depend on the following:

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 or metastatic.

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.

The three ways that cancer spreads in the body are:

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

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 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 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 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 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 replacing blood -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 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.

Kinase inhibitor therapy is another type of targeted therapy being studied in the treatment of recurrent Ewing 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

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 child's doctor for clinical trials that are not listed here but may be right for your child.

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:

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 giving combination 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 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.

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:

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. Chemotherapy followed by radiation therapy or stem cell transplant is being studied for Ewing sarcoma that has spread to the lung only.

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.

Recurrent Ewing Sarcoma

Treatment of recurrent Ewing sarcoma may include the following:

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.

To Learn More About Ewing Sarcoma

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

For more childhood cancer information and other general cancer resources, see the following:

Changes to This Summary (01 / 28 / 2013)

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.

Editorial changes were made to this summary.

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About PDQ

PDQ is a comprehensive cancer database available on NCI's Web site.

PDQ is the National Cancer Institute's (NCI's) comprehensive cancer information database. Most of the information contained in PDQ is available online at NCI's Web site. PDQ is provided as a service of the NCI. The NCI is part of the National Institutes of Health, the federal government's focal point for biomedical research.

PDQ contains cancer information summaries.

The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries are available in two versions. The health professional versions provide detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions provide current and accurate cancer information.

Images in the PDQ summaries are used with permission of the author(s), artist, and/or publisher for use within the PDQ summaries only. Permission to use images outside the context of PDQ information must be obtained from the owner(s) and cannot be granted by the National Cancer Institute. Information about using the illustrations in the PDQ summaries, along with many other cancer-related images, are available in Visuals Online, a collection of over 2,000 scientific images.

The PDQ cancer information summaries are developed by cancer experts and reviewed regularly.

Editorial Boards made up of experts in oncology and related specialties are responsible for writing and maintaining the cancer information summaries. The summaries are reviewed regularly and changes are made as new information becomes available. The date on each summary ("Date Last Modified") indicates the time of the most recent change.

PDQ also contains information on clinical trials.

A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." In the United States, about two-thirds of children with cancer are treated in a clinical trial at some point in their illness.

Listings of clinical trials are included in PDQ and are available online at NCI's Web site. Descriptions of the trials are available in health professional and patient versions. For additional help in locating a childhood cancer clinical trial, call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237).

The PDQ database contains listings of groups specializing in clinical trials.

The Children's Oncology Group (COG) is the major group that organizes clinical trials for childhood cancers in the United States. Information about contacting COG is available on the NCI Web site or from the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237).

Last Revised: 2013-01-28


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