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\nEwing sarcoma most often forms in the bones of the legs, arms, feet, hands, chest, pelvis, spine, or skull. Less often, it forms in the soft tissue of the trunk, arms, legs, head, neck, retroperitoneum (area in the back of the abdomen behind the tissue that lines the abdominal wall and covers most of the organs in the abdomen), or other areas.
\nEwing sarcoma is most common in adolescents and young adults (teens through mid-20s).
\nEwing sarcoma has also been called peripheral primitive neuroectodermal tumor, Askin tumor (Ewing sarcoma of the chest wall), extraosseous Ewing sarcoma (Ewing sarcoma in tissue other than bone), and Ewing sarcoma family of tumors.
\nUndifferentiated small round cell sarcoma usually forms in the bones or the muscles that are attached to bones and that help the body move. There are five types of undifferentiated small round cell sarcoma that are treated like Ewing sarcoma:
\nAnything that increases a person's chance of getting a disease is called a risk factor. Not every child with one or more of these risk factors will develop Ewing sarcoma or other sarcomas, and they can develop in some children who don't have any known risk factors. Children with Fanconi anemia may be at increased risk for Ewing sarcoma. Talk with your child's doctor if you think your child may be at risk.
\nThese and other signs and symptoms may be caused by Ewing sarcoma or by other conditions. Check with your child\u2019s doctor if they have any of the following:
\nProcedures that make pictures of the bones and soft tissues and nearby areas help diagnose Ewing sarcoma and show how far the cancer has spread. The process used to find out if cancer cells have spread within and around the bones and soft tissues or to other parts of the body is called staging.
\nTo plan treatment, it is important to know whether the cancer has spread to other parts of the body. Tests and procedures to detect, diagnose, and stage Ewing sarcoma are usually done at the same time.
\nIn addition to asking about your child's personal and family health history and doing a physical exam, your child's doctor may perform the following tests and procedures to diagnose or stage Ewing sarcoma:
\nTissue samples are removed during a 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. The following types of biopsy are used to diagnose Ewing sarcoma:
\nThe specialists (pathologist, radiation oncologist, and surgeon) who will treat the patient usually work together to decide the best site to place the needle or biopsy incision. The selection of the biopsy site is important. A biopsy site that is not properly selected may result in more extensive surgery to remove the tumor or a larger area that is treated with radiation therapy.
\nIf 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.
\nThe following tests may be done on the tissue that is removed:
\nThe factors that affect prognosis are different before and after treatment.
\nBefore any treatment is given, prognosis depends on:
\nAfter treatment is given, prognosis is affected by:
\nIf the cancer recurs after initial treatment, prognosis depends on:
\nThe 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 and stage Ewing sarcoma are used to help make decisions about treatment. Treatment is based on whether the cancer is localized, metastatic, or recurrent.
\nThe cancer is found in the bone or soft tissue where it began and may have spread to nearby tissue, including nearby lymph nodes.
\nThe cancer has spread from the bone or soft tissue where 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.
\nThe cancer has recurred (come back) after it has been treated. The cancer may come back in the bone or soft tissue where it began or in another part of the body.
\nCancer can spread through tissue, the lymph system, and the blood:
\nWhen 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.
\nThe 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.
\nDifferent 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.
\nBecause cancer in children and adolescents is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment.
\nTreatment 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:
\nChemotherapy 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). Combination chemotherapy is treatment using more than one anticancer drug.
\nSystemic combination chemotherapy is part of the treatment for all patients with Ewing tumors. It is often the first treatment given and lasts for about 6 to 12 months. Chemotherapy is often given to shrink the tumor before surgery or radiation therapy and to kill any tumor cells that may have spread to other parts of the body.
\nFor more information, see Drugs Approved for Soft Tissue Sarcoma.
\nRadiation 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. External radiation therapy uses a machine outside the body to send radiation toward the area of the body with cancer.
\nRadiation therapy is used when the tumor cannot be removed by surgery or when surgery to remove the tumor will affect important body functions or the way the child will look. It may be 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 any tumor that remains after surgery and tumors that have spread to other parts of the body.
\nRadiation therapy may also be used as palliative therapy to relieve symptoms caused by the tumor in the bone.
\nSurgery 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.
\nAfter the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy.
\nHigh doses of chemotherapy are given to kill cancer cells. Healthy cells, including blood-forming cells, are also destroyed by the cancer treatment. Stem cell transplant is a treatment to replace the blood-forming cells. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient and are frozen and stored. After the patient completes chemotherapy, 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. Chemotherapy with stem cell rescue is used to treat localized and recurrent Ewing sarcoma.
\nThis 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 website.
\nImmunotherapy helps a person's immune system fight cancer. Types of immunotherapy include:
\nTo learn more about side effects that begin during treatment for cancer, visit Side Effects.
\nSide effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include the following:
\nSome 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. For more information, see Late Effects of Treatment for Childhood Cancer.
\nFor 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.
\nMany 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.
\nPatients 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.
\nSome 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.
\nClinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI\u2019s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.
\nAs your child goes through treatment, they will have follow-up tests or checkups. Some tests that were done to diagnose or stage the cancer may be repeated 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. These tests are sometimes called follow-up tests or check-ups.
\nSome 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).
\nFor information about the treatments listed below, see the Treatment Option Overview section.
\nStandard treatments for newly diagnosed localized Ewing sarcoma include:
\nUse our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.
\nFor information about the treatments listed below, see the Treatment Option Overview section.
\nStandard treatments for newly diagnosed metastatic Ewing sarcoma include:
\nUse our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.
\nFor information about the treatments listed below, see the Treatment Option Overview section.
\nThere is no standard treatment for recurrent Ewing sarcoma but treatment options may include the following:
\nUse our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.
\nFor more information from the National Cancer Institute about Ewing sarcoma, see the following:
\nFor more childhood cancer information and other general cancer resources, visit:
\nPhysician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. 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 come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish.
\nPDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government\u2019s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH.
\nThis PDQ cancer information summary has current information about the treatment of childhood Ewing sarcoma. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care.
\nEditorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary (\"Updated\") is the date of the most recent change.
\nThe information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Pediatric Treatment Editorial Board.
\nA 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.\" Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
\nClinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
\nPDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as \u201cNCI\u2019s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].\u201d
\nThe best way to cite this PDQ summary is:
\nPDQ\u00ae Pediatric Treatment Editorial Board. PDQ Ewing Sarcoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/bone/patient/ewing-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389350]
\nImages in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
\nThe information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page.
\nMore information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website\u2019s E-mail Us.
\nUpdated:
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