ON THIS PAGE: You will find information about the estimated number of children and teens who will be diagnosed with Ewing sarcoma each year. You will also read general information on surviving the disease. Remember, survival rates depend on several factors, and no 2 people with a tumor are the same. Use the menu to see other pages.

Every person is different, with different factors influencing their risk of being diagnosed with this tumor and the chance of recovery after a diagnosis. It is important to talk with your doctor about any questions you have around the general statistics provided below and what they may mean for your child individually. The original sources for these statistics are provided at the bottom of this page.

How many children and teens are diagnosed with Ewing sarcoma?

Each year, about 200 children and teens in the United States are diagnosed with a Ewing sarcoma. This type of tumor makes up 1% of all cancers in children and adolescents younger than 15 and 2% of all cancers in teens aged 15 to 19. These tumors can also affect younger children and young adults in their 20s and 30s. Almost all cases of Ewing sarcoma occur in White people and Hispanic people.

What is the survival rate for Ewing sarcoma?

There are different types of statistics that can help doctors evaluate a child or teen’s chance of recovery from Ewing sarcoma. These are called survival statistics. A specific type of survival statistic is called the relative survival rate. It is often used to predict how having a tumor may affect life expectancy. Relative survival rate looks at how likely people with Ewing sarcoma are to survive for a certain amount of time after their initial diagnosis or start of treatment compared to the expected survival of similar people without this tumor.

Example: Here is an example to help explain what a relative survival rate means. Please note this is only an example and not specific to this type of cancer. Let’s assume that the 5-year relative survival rate for a specific type of cancer is 90%. “Percent” means how many out of 100. Imagine there are 1,000 people without cancer, and based on their age and other characteristics, you expect 900 of the 1,000 to be alive in 5 years. Also imagine there are another 1,000 people similar in age and other characteristics as the first 1,000, but they all have the specific type of cancer that has a 5-year survival rate of 90%. This means it is expected that 810 of the people with the specific cancer (90% of 900) will be alive in 5 years.

It is important to remember that statistics on the survival rates for children and teens with Ewing sarcoma are only an estimate. They cannot tell an individual person if the tumor will or will not shorten their life. Instead, these statistics describe trends in groups of people previously diagnosed with the same disease, including specific stages of the disease.

The survival rates for Ewing sarcoma vary based on several factors. These include the stage of tumor, a person’s age and general health, and how well the treatment plan works. There are other factors that have been linked to higher survival rates for all stages of Ewing tumors. These factors include being younger than 10, having a smaller sized tumor, having a tumor located in an arm or leg, and having normal levels of the enzyme lactate dehydrogenase (LDH).

Based on age, the overall 5-year relative survival rate for this type of cancer in the United States is 78% for children younger than 15 and 64% for teens ages 15 to 19.

The overall 5-year relative survival rate for people with Ewing sarcoma is 63%. If the tumor is found only in the area it began (called localized), the 5-year relative survival rate is 82%. If it has spread to the nearby region (called regional), the 5-year relative survival rate is 71%. If the tumor has spread to distant areas at the time of diagnosis (called metastasis), the 5-year relative survival rate is 39%.

Experts measure relative survival rate statistics for Ewing sarcoma every 5 years. This means the estimate may not reflect the results of advancements in how Ewing sarcoma is diagnosed or treated from the last 5 years. Talk with your child’s doctor if you have any questions about this information. Learn more about understanding statistics.

Statistics adapted from the websites of the American Cancer Society and St. Jude Children’s Research Hospital. Additional source was Seigel R, et al.: Cancer Statistics 2023. CA: A Cancer Journal for Clinicians. 2023 Jan; 73(1):17–48. doi/full/10.3322/caac.21763. (All sources accessed February 2023.)

The next section in this guide is Medical Illustrations. It offers drawings of the major bones in the body. Use the menu to choose a different section to read in this guide.

ON THIS PAGE: You will find out more about the factors that increase the chance of developing Ewing sarcoma. Use the menu to see other pages.

What are the risk factors for Ewing sarcoma?

A risk factor is anything that increases a person’s chance of developing cancer. Although risk factors often influence the development of cancer, most do not directly cause cancer. Some people with several risk factors never develop cancer, while others with no known risk factors do.

Doctors and researchers do not know what causes most cancers in children and young adults. But the following factors may raise a person’s chance of developing Ewing sarcoma:

• Genetic changes. Changes in a tumor cell's chromosomes appear to be responsible for Ewing sarcoma, but the disease is not inherited. This means that it isn’t passed down from a parent to a child. The genetic changes occur for no known reason.

  A high percentage of Ewing sarcoma cells have a chromosomal translocation, which means that small pieces of genetic material have swapped places inside the tumor cell. The translocation is usually between chromosomes 11 and 22, although it may also occur between chromosomes 21 and 22, 7 and 22, and 17 and 22. The fusion of these bits of genetic material results in the out-of-control growth of Ewing sarcoma cells.

• Age. Ewing sarcoma can occur at any age. But more than half (50%) of people with Ewing sarcoma are between the ages of 10 and 20, with a median age of 15.

• Sex. Ewing sarcoma is more common in boys than girls.

• Race/ethnicity. Ewing sarcoma occurs most frequently in White people and is rare in Black people in the United States and Africa. Ewing sarcoma has been reported in Japan and is uncommon in China. Recent research suggests that there are 3 susceptibility loci, which are regions of a chromosome that are inherited with an illness, for Ewing sarcoma (1p36.22, 10q21, and 15q15). A polymorphism has been identified in the EGFR2 gene, which may cooperate with and magnify the enhanced activity of the EWSR1-FLI1 fusion gene product. The polymorphism associated with the increased risk for Ewing sarcoma is found at a higher frequency in White people than in Black people or Asian people, which could explain the difference in frequency of the disease in these populations.

The next section in this guide is Symptoms and Signs. It explains what changes or medical problems Ewing sarcoma can cause. Use the menu to choose a different section to read in this guide.

ON THIS PAGE: You will find a list of common tests, procedures, and scans that doctors use to find the cause of a medical problem. Use the menu to see other pages.

Doctors use many tests to find, or diagnose, cancer. They also do tests to learn if cancer has spread to another part of the body from where it started. If the cancer has spread, it is called metastasis. Doctors may also do tests to learn which treatments could work best.

For most types of cancer, a biopsy is the only sure way for the doctor to know if an area of the body has cancer. In a biopsy, the doctor takes a small sample of tissue for testing in a laboratory. If a biopsy is not possible, the doctor may suggest other tests that will help make a diagnosis.

How Ewing sarcoma is diagnosed

There are different tests used for diagnosing Ewing sarcoma. Not all tests described here will be used for every person. The doctor may consider these factors when choosing a diagnostic test:

• The type of cancer suspected

• Signs and symptoms

• Age and general health

• The results of earlier medical tests

In addition to a physical examination, the following tests may be used to help diagnose Ewing sarcoma:

• Blood tests. A complete blood count (CBC) is a blood test that counts the number of each type of blood cell. Abnormal levels of white blood cells, red blood cells, and platelets can be a sign that the tumor has spread. The doctor may also check liver and kidney function and look for high levels of a particular blood enzyme called lactate dehydrogenase, or LDH, which sometimes helps signal the presence of a tumor in the body. While a CBC alone cannot make the diagnosis of Ewing sarcoma, it can help doctors to diagnose the disease. 

Imaging tests

Imaging tests show pictures of the inside of the body. They can show if cancer has spread.

• X-ray. An x-ray creates a picture of the organs and tissues inside of the body using a small amount of radiation. Doctors can usually find bone tumors with an x-ray.

• Computed tomography (CT or CAT) scan. A CT scan takes pictures of the inside of the body using x-rays taken from different angles. A computer combines these pictures into a detailed, 3-dimensional image that shows any abnormalities or tumors. For Ewing sarcoma, a CT scan of the chest will be done to see if the tumor has spread to the lungs. A CT scan can be used to measure the tumor’s size. Sometimes, a special dye called a contrast medium is given before the scan to provide better detail on the image. This dye can be injected into a patient’s vein or given as a pill or liquid to swallow.

• Magnetic resonance imaging (MRI). An MRI produces detailed images of the inside of the body using magnetic fields, not x-rays. MRI can be used to measure the tumor’s size. A special dye called a contrast medium is given before the scan to create a clearer picture. This dye can be injected into a patient’s vein or given as a pill or liquid to swallow. MRI is more helpful than a CT scan in revealing where the tumor is in relation to nearby nerves and blood vessels.

• Positron emission tomography (PET) or PET-CT scan. A PET scan is a way to create pictures of organs and tissues inside the body. A PET scan is usually combined with a CT scan (see above), called a PET-CT scan. However, you may hear the doctor refer to this procedure just as a PET scan. A small amount of a radioactive sugar substance is injected into the patient’s body. This sugar substance is taken up by cells that use the most energy. Because cancer tends to use energy actively, it absorbs more of the radioactive substance. However, the amount of radiation in the substance is too low to be harmful. A scanner then detects this substance to produce images of the inside of the body. For Ewing sarcoma, an integrated PET-CT scan is often more sensitive than a PET scan alone or a bone scan.

• Bone scan. A bone scan looks at the inside of the bones using a radioactive tracer. The amount of radiation in the tracer is too low to be harmful. The tracer is injected into a patient’s vein. It collects in areas of the bone and is detected by a special camera. Healthy bone appears lighter to the camera, and areas of injury, such as those caused by a tumor, stand out on the image.

Surgical tests

• Biopsy. A biopsy is the only way to make a definite diagnosis, even if other tests can suggest that cancer is present. During biopsy, a small amount of tissue is removed for examination under a microscope. If the tumor is in an arm or leg, an orthopedic oncologist or an interventional radiologist who has experience with Ewing sarcoma should perform the biopsy. An orthopedic oncologist is a doctor who specializes in cancers of the musculoskeletal system. An interventional radiologist is a doctor who is trained in both radiology and minimally invasive surgery.

  During the procedure, the doctor may take a sample of the tumor, bone marrow (see below), or both. A pathologist analyzes the sample(s) removed during the biopsy. A pathologist is a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease.

• Bone marrow aspiration and biopsy. These 2 procedures are done to examine the bone marrow and see if Ewing sarcoma is present. Bone marrow has both a solid and a liquid part. A bone marrow aspiration removes a sample of the fluid with a needle. A bone marrow biopsy is the removal of a small amount of solid tissue using a needle. These procedures are often done at the same time and may be called a bone marrow examination.

  A pathologist then analyzes the sample(s). A common site for a bone marrow aspiration and biopsy is the pelvic bone, which is located in the lower back by the hip. Doctors often give a type of medication called anesthesia beforehand to numb the area. Anesthesia is medication that blocks the awareness of pain. Stronger types of anesthesia can also be used to lessen the pain. If a patient with Ewing sarcoma receives a PET scan and there are no other sites of disease, then a bone marrow aspiration and biopsy may not need to be done, since the incidence of bone marrow involvement as the only metastatic site is very low.

Other laboratory tests

Other laboratory tests may be done on the tissue sample(s) removed during a biopsy to learn more about the tumor.

• Immunohistochemistry. This test finds out whether there are Ewing sarcoma cells in the tissue sample.

• Cytogenetic tests and reverse transcription polymerase chain reaction (RT-PCR). These tests find out if the genetic changes that characterize Ewing sarcoma cells are present in the sample.

• Biomarker testing of the tumor. Your child’s doctor may recommend running laboratory tests on a tumor and/or bone marrow sample to identify specific genes, proteins, and other factors unique to the tumor. This may also be called molecular testing of the tumor. Results of these tests can help determine your child’s treatment options.

After diagnostic tests are done, the doctor will review the results with you and your child. If the diagnosis is cancer, these results also help the doctor describe the cancer. This is called staging.

The next section in this guide is Stages. It explains the system doctors use to describe the extent of the disease. Use the menu to choose a different section to read in this guide.

ON THIS PAGE: You will learn about the different types of treatments doctors use for children and young adults with Ewing sarcoma. Use the menu to see other pages.

In general, cancer in children and young adults is uncommon. This means it can be hard for doctors to plan treatments unless they know what has been most effective in this age group. That is why more than 60% of children and teens with cancer are treated as part of a clinical trial. A clinical trial is a research study that tests a new approach to treatment. The “standard of care” is the best treatments known based on previous clinical trials. Clinical trials may test approaches such as a new drug, a new combination of existing treatments, or new doses of current therapies. The health and safety of all people participating in clinical trials are closely monitored.

To take advantage of these newer treatments, children and teens with Ewing sarcoma should be treated at a specialized cancer center. Doctors at these centers have extensive experience in treating this age group and have access to the latest research. A doctor who specializes in treating children and teens with cancer is called a pediatric oncologist. If a pediatric cancer center is not nearby, general cancer centers sometimes have pediatric specialists who are able to be part of your child’s care.

How Ewing sarcoma is treated

In many cases, a team of doctors works with a child and the family to provide care. This is called a multidisciplinary team. Pediatric cancer centers often have extra support services for children and teens and their families, such as child life specialists, dietitians, physical therapists, occupational therapists, social workers, and counselors. Special activities and programs to help your family cope may also be available. Learn more about the clinicians who provide cancer care.

It is important that patients are seen by medical experts from different specialties across medical disciplines who are experienced with treatment of Ewing sarcoma from the time of diagnosis. This includes a:

• Radiologist. A doctor trained in diagnosing and treating cancer using medical imaging.

• Medical oncologist. A doctor trained to treat cancer with systemic treatments using medications.

• Pediatric oncologist. A doctor trained to treat cancer in children and teens.

• Pathologist. A doctor who diagnoses disease by explaining laboratory tests and evaluating cells, tissues, and organs.

• Surgical or orthopedic oncologist. A doctor trained to treat cancer using surgery.

• Radiation oncologist. A doctor trained to treat cancer with radiation therapy. This doctor will be part of the team if radiation therapy is recommended.

It is extremely important that the biopsy incision (see Diagnosis) is placed in the best location. Therefore, the surgical or orthopedic oncologist who will perform the surgery should be involved in the decision regarding where the biopsy incision will be made. This is particularly important if the tumor can be totally removed or, if the tumor is located in an arm or leg, whether a limb-sparing procedure can be done.

Children and young adults with Ewing sarcoma should be treated in clinical trials specifically designed for their disease. A typical treatment plan for Ewing sarcoma includes systemic therapy with medication that treats the entire body, such as chemotherapy, combined with localized therapy. Systemic therapy is the use of medication to destroy cancer cells. Localized therapy focuses on treating the tumor itself using surgery, radiation therapy, or both. When more than 1 treatment is used, it is called combination therapy.

Doctors make treatment recommendations based on the stage of the disease and the age of the patient, while trying to avoid or reduce long-term side effects of treatment.

Take time to learn about all of the treatment options and be sure to ask questions about things that are unclear. Talk with the doctor about the goals of each treatment and what your child can expect while receiving the treatment. These types of conversations are called “shared decision-making.” Shared decision-making is when you and your child's doctors work together to choose treatments that fit the goals of your child’s care. Shared decision-making is important for Ewing sarcoma because there are different treatment options. Learn more about making treatment decisions.

The common types of treatment used for Ewing sarcoma are described below. The care plan may also include treatment for symptoms and side effects, an important part of cancer care. Learn more about preparing your child for treatment.

READ MORE BELOW:

• Chemotherapy

• Surgery

• Radiation therapy

• Bone marrow transplantation/stem cell transplantation

• Physical, emotional, social, and financial effects of cancer

• Remission and the chance of recurrence

• If treatment does not work

Chemotherapy

Chemotherapy is the use of drugs to destroy cancer cells, usually by keeping the cancer cells from growing, dividing, and making more cells. Chemotherapy is given by a pediatric or medical oncologist, a doctor who specializes in treating cancer with medication. Some patients may receive chemotherapy in their doctor’s office, while others may go to a hospital or outpatient clinic.

Systemic chemotherapy gets into the bloodstream to reach cancer cells throughout the body. Common ways to give systemic chemotherapy include an intravenous (IV) tube placed into a vein using a needle or as a pill or capsule that is swallowed (orally). However, chemotherapy for Ewing sarcoma is usually injected into a vein or muscle; it is rarely given by mouth.

When possible, treatment for Ewing sarcoma begins with chemotherapy. After this first chemotherapy is finished, the doctor may use localized surgery or radiation therapy (see below) followed by more chemotherapy to get rid of any remaining cancer cells. When chemotherapy is used before another treatment, it is called “neoadjuvant chemotherapy.” Other times, the doctor may use surgery or radiation therapy first and then give chemotherapy after. This is called “adjuvant chemotherapy.”

A chemotherapy regimen, or schedule, usually consists of a specific number of cycles given over a set period of time. A patient may receive 1 drug at a time or combinations of different drugs given at the same time. Children and young adults with Ewing sarcoma should receive cyclophosphamide (Cytoxan, Neosar), doxorubicin (Adriamycin), etoposide (Toposar, VePesid), ifosfamide (Ifex), and/or vincristine (Oncovin, Vincasar PFS). For Ewing sarcoma that has not spread to other parts of the body, the standard schedule is chemotherapy every 2 weeks. Patients with metastatic Ewing sarcoma may also be treated with the above medications and dactinomycin (Cosmegen).

The side effects of chemotherapy depend on the individual and the dose used, but they can include fatigue, risk of infection, nausea and vomiting, hair loss, loss of appetite, and diarrhea. These side effects usually go away after treatment is finished.

Children and young adults receiving chemotherapy for Ewing sarcoma may be at risk for developing neutropenia, which is an abnormally low level of a type of white blood cell called neutrophils. All white blood cells help the body fight infection. The doctor may give the patient medications to increase their white blood cell levels. These medications are called white blood cell growth factors, also known as colony-stimulating factors (CSFs). Treating neutropenia is an important part of the overall treatment plan.

In addition, parents of children and young adults receiving chemotherapy for Ewing sarcoma should talk with the doctor about fertility preservation, including sperm storage, before chemotherapy starts.

Learn more about the basics of chemotherapy.

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Surgery

Surgery is the removal of the tumor and some surrounding healthy tissue during an operation. An orthopedic oncologist is usually the doctor who will perform the surgery.

When possible, surgical removal of the tumor should happen after chemotherapy. Surgery may also be needed to remove any remaining cancer cells after chemotherapy or radiation therapy.

Often, a tumor can be removed without causing disability. However, if the tumor occurs in an arm or leg, surgery to remove much of the bone may affect the limb’s ability to function. Bone grafts from other parts of the body may help reconstruct the limb, and a prosthesis made of metal or plastic bones or joints can replace lost tissue. Physical therapy after surgery can help children and young adults learn to use the limb again. Support services are available to help patients cope with the emotional effects of the loss of a limb. Learn more about rehabilitation services.

Before surgery, talk with the health care team about possible side effects from the specific surgery your child will have. Learn more about the basics of cancer surgery.

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Radiation therapy

Radiation therapy is the use of high-energy x-rays or other high-energy particles to destroy tumor cells. A doctor who specializes in giving radiation therapy to treat cancer is called a radiation oncologist. For Ewing sarcoma, radiation therapy is used when surgery is not possible or if surgery did not remove all of the tumor cells, as well as when chemotherapy was not effective.

The most common type of radiation treatment is called external-beam radiation therapy, which is radiation given from a machine outside the body. When radiation therapy is given using implants, it is called internal radiation therapy or brachytherapy. A radiation therapy regimen, or schedule, usually consists of a specific number of treatments given over a set period of time. Intraoperative radiation therapy, which is given inside the body during surgery, is being studied in clinical trials.

Side effects from radiation therapy may include fatigue, mild skin reactions, upset stomach, and loose bowel movements. Most side effects go away soon after treatment is finished. In the long term, radiation therapy can also interfere with normal bone growth and increase the risk of developing a secondary cancer. Learn more about the basics of radiation therapy.

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Bone marrow transplant/stem cell transplant

For Ewing sarcoma, a bone marrow transplant is an approach that is still being studied to find out if it is an effective treatment option. It should only be done as part of a clinical trial.

A bone marrow transplant is a medical procedure in which bone marrow that contains cancer is replaced by highly specialized cells. These cells, called hematopoietic stem cells, develop into healthy bone marrow. Hematopoietic stem cells are blood-forming cells found both in the bloodstream and in the bone marrow. This procedure is also called a stem cell transplant or hematopoietic stem cell transplant.

Before recommending a transplant, doctors will talk with the patient and family about the risks of this treatment. They will also consider several other factors, such as the type of tumor, results of any previous treatment, and the child’s age and general health.

There are 2 types of hematopoietic stem cell transplantation, depending on the source of the replacement blood stem cells: allogeneic (ALLO) and autologous (AUTO). ALLO uses donated stem cells, while AUTO uses the person’s own stem cells. AUTO transplants are the type used most commonly to treat Ewing sarcoma.

The goal of stem cell transplantation is to destroy all of the cancer cells in the marrow, blood, and other parts of the body using high doses of chemotherapy and/or radiation therapy, and then allow replacement blood stem cells to create healthy bone marrow.

Side effects depend on the type of transplant, the person's general health, and other factors. Learn more about the basics of bone marrow and stem cell transplantation.

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Physical, emotional, social, and financial effects of cancer

Cancer and its treatment cause physical symptoms and side effects, as well as emotional, social, and financial effects. Managing all of these effects is called palliative and supportive care. It is an important part of your child’s care that is included along with treatments intended to slow, stop, or eliminate the cancer.

Palliative and supportive care focuses on improving how your child feels during treatment by managing symptoms and supporting patients and their families with other, non-medical needs. Any person, regardless of age or type and stage of cancer, may receive this type of care. And it often works best when it is started right after a cancer diagnosis. People who receive palliative and supportive care along with treatment for the cancer often have less severe symptoms, better quality of life, and report that they are more satisfied with treatment.

Palliative treatments vary widely and often include medication, nutritional changes, relaxation techniques, emotional and spiritual support, and other therapies. Your child may also receive palliative treatments, such as chemotherapy, surgery, or radiation therapy, to improve symptoms.

Before treatment begins, talk with your child’s doctor about the goals of each treatment in the recommended treatment plan. You should also talk about the possible side effects of the specific treatment plan and palliative and supportive care options. Many patients also benefit from talking with a social worker and participating in support groups. Ask your doctor about these resources, too.

Cancer care is often expensive, and navigating health insurance can be difficult. Ask your doctor or another member of your health care team about talking with a financial navigator or counselor who may be able to help with your financial concerns.

During treatment, your child’s health care team may ask you to answer questions about your child’s symptoms and side effects and to describe each problem. Be sure to tell the health care team if your child is experiencing a problem. This helps the health care team treat any symptoms and side effects as quickly as possible. It can also help prevent more serious problems in the future.

Learn more about the importance of tracking side effects in another part of this guide. Learn more about palliative and supportive care in a separate section of this website.

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Remission and the chance of recurrence

A remission is when cancer cannot be detected in the body and there are no symptoms. This may also be called having “no evidence of disease” or NED.

A remission may be temporary or permanent. This uncertainty causes many people to worry that the cancer will come back. While many remissions are permanent, it is important to talk with the doctor about the possibility of the cancer returning. Understanding the risk of recurrence and the treatment options may help everyone feel more prepared if the cancer does return. Learn more about coping with the fear of recurrence.

If the cancer returns after the original treatment, it is called recurrent disease. It may come back in the same place (called a local recurrence), nearby (regional recurrence), or in another place (distant recurrence). When it occurs, recurrence is most common within the first 2 years after Ewing sarcoma treatment has finished. However, it is also important to note that late recurrences that develop up to 5 years after treatment are more common with Ewing sarcoma than with other types of cancers in this age group.

If a recurrence happens, a new cycle of testing will begin to learn as much as possible about it. After this testing is done, the doctor will talk about the treatment options with you. The next round of treatment will depend on where and when the cancer recurred and how it was first treated. The doctor may recommend chemotherapy, including cyclophosphamide (available as a generic drug) and irinotecan (Camptosar), temozolomide (Temodar), and topotecan (Hycamtin, Brakiva); radiation therapy; and/or surgery to remove new tumors. Bone marrow/stem cell transplantation may also be recommended.

The doctor may suggest clinical trials that are studying new ways to treat recurrent Ewing sarcoma. Or, they may suggest having the tumor analyzed for changes in genes that can be targeted by specific drugs. Whichever treatment plan you choose, palliative and supportive care will be important for relieving symptoms and side effects.

People with recurrent Ewing sarcoma and their family sometimes experience emotions such as disbelief or fear. You are encouraged to talk with your child's health care team about these feelings and ask about support services to help your family cope. Learn more about dealing with a recurrence.

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If treatment does not work

Although treatment is successful for many children and young adults with cancer, sometimes it is not. If the Ewing sarcoma cannot be cured or controlled, this is called advanced or terminal disease.

This diagnosis is stressful, and advanced cancer may be difficult to discuss. However, it is important to have open and honest conversations with the health care team to express your family’s feelings, preferences, and concerns. The health care team has special skills, experience, and knowledge to support patients and their families and is there to help.

Hospice care is designed to provide the best possible quality of life for people who are expected to live less than 6 months. Parents and guardians are encouraged to talk with the health care team about hospice options, which include hospice care at home, a special hospice center, or other health care locations. Nursing care and special equipment can make staying at home a workable option for many families. Some children and young adults may be happier and more comfortable if they can attend school part-time or keep up other activities and social connections. The health care team can help parents or guardians decide on an appropriate level of activity. Making sure the patient is physically comfortable and free from pain is extremely important as part of end-of-life care. Learn more about caring for a terminally ill child and advanced cancer care planning.

The death of a child is an enormous tragedy, and families may need support to help them cope with the loss. Pediatric cancer centers often have professional staff and support groups to help with the process of grieving. Learn more on grieving the loss of a child.

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The next section in this guide is About Clinical Trials. It offers more information about research studies that are focused on finding better ways to care for children and young adults with cancer. Use the menu to choose a different section to read in this guide.

ON THIS PAGE: You will read about the scientific research being done to learn more about Ewing sarcoma and how to treat it. Use the menu to see other pages.

Doctors are working to learn more about Ewing sarcoma, how to best treat it, and how to provide the best care to people diagnosed with this disease. The following areas of research may include new options for patients through clinical trials. Always talk with the doctor about the best diagnostic and treatment options for your child.

• Allogeneic (ALLO) stem cell transplantation. In an ALLO bone marrow/stem cell transplant (see Types of Treatment for a description), the patient is treated with high doses of chemotherapy, radiation therapy, or both to destroy as many tumor cells as possible and to prevent the patient’s immune system from rejecting the donated stem cells. After the high-dose therapy is given, the stem cells obtained from a healthy donor, usually a sibling, are infused into the patient's bloodstream. ALLO stem cell transplants combined with sirolimus (Rapamune) and other drugs have been shown to prevent the growth of Ewing cells in the laboratory.

• Bilateral lung radiation. For all patients with disease that has spread to the lung, whole-lung radiation therapy should be considered, even if chemotherapy has removed most signs of metastatic disease. During this treatment, radiation therapy is given to both lungs after the patient has completed chemotherapy. For some patients, autologous (AUTO) bone marrow/stem cell transplant (see Types of Treatment for a description) is recommended but should only be done in a clinical trial.

• Treatment for recurrent disease. Some studies focused on recurrent Ewing sarcoma are evaluating tumor DNA to look for molecular changes that can be targeted during treatment. This is done using a technology called next-generation sequencing.

• Circulating tumor DNA. Circulating tumor DNA (ctDNA) is cancer DNA found in the blood. Researchers are looking at the benefits of assessing ctDNA at the time of diagnosis and after 2 cycles of chemotherapy.

• Palliative and supportive care. Clinical trials are underway to find better ways of reducing symptoms and side effects of current Ewing sarcoma treatments to improve comfort and quality of life for patients.

• Research to better understand Ewing sarcoma. In addition to the consistent aberrations involving the EWSR1 gene at 22q12, additional numerical and structural aberrations have been observed in Ewing sarcoma, including gains of chromosomes 2, 5, 8, 9, 12, and 15; the nonreciprocal translocation t(1;16)(q12;q11.2); and deletions on the short arm of chromosome 6. Trisomy 20 may also be associated with a more aggressive subset of Ewing sarcoma. Other recurring genomic alterations identified in several genes in Ewing sarcoma are STAG2 mutations (seen in 15% to 20% of cases), which is associated with advanced stage disease; CDKN2A deletions (seen in 12% to 22% of cases); and TP53 mutations (seen in 6% to 7% of cases), which, when present with STAG2 mutations, are associated with a poor outcome. Trisomy 8 is the most frequent chromosomal alteration in Ewing sarcoma and occurs in about 50% of tumors. Gain of 1q and loss of chromosome 16q occurs in about 20% of patients and often occur together.

• Research in fusion oncoproteins. Fusion oncoproteins are abnormal molecules made of the fused parts of 2 proteins. The National Cancer Institute launched the Fusion Oncoproteins in Childhood Cancers (FusOnC2) Consortium to learn more about the role of fusion oncoproteins in childhood cancers.

Looking for More About the Latest Research?

If you would like more information about the latest areas of research in Ewing sarcoma, explore these related items that will take you outside of this guide:

• To find clinical trials specific to the diagnosis, talk with the doctor or search online clinical trial databases.

• Visit the Cancer.Net Blog to review research in Ewing sarcoma presented at the 2022 ASCO Annual Meeting.

• Get updates from Cancer.Net delivered right to your inbox. Subscribe to the Inside Cancer.Net email newsletter.

• Visit the website of Conquer Cancer, the ASCO Foundation, to find out how to help support cancer research. Please note that this link takes you to a different ASCO website.

The next section in this guide is Coping with Treatment. It offers some guidance on how to cope with the physical, emotional, social, and financial changes that cancer and its treatment can bring. Use the menu to choose a different section to read in this guide.

ON THIS PAGE: You will read about your child's medical care after cancer treatment is completed and why this follow-up care is important. Use the menu to see other pages.

Care for people diagnosed with cancer does not end when active treatment has finished. The health care team will continue to check that the cancer has not come back, manage any side effects, and monitor your child’s overall health. This is called follow-up care. Everyone treated for cancer, including Ewing sarcoma, should have life-long, follow-up care by a doctor or health care team familiar with the late effects of chemotherapy and radiation therapy.

This follow-up care may include regular physical examinations, medical tests, or both. Doctors want to keep track of your child's recovery in the months and years ahead. Based on the type of treatment your child received, the doctor will determine what examinations and tests are needed to check for long-term side effects and the possibility of secondary cancers. Follow-up care for Ewing sarcoma usually includes bone scans, computed tomography (CT) scans, magnetic resonance imaging (MRI) scans, and x-rays. The doctor can recommend which screening tests are necessary and how often they should be done.

Cancer rehabilitation may be recommended, and this could mean any of a wide range of services such as physical therapy, occupational therapy, family or individual counseling, nutritional planning, and/or educational assistance. The goal of rehabilitation is to help survivors and their families regain control over many aspects of their lives and remain as independent as possible. Learn more about cancer rehabilitation.

Learn more about the importance of follow-up care.

Watching for recurrence

One goal of follow-up care is to check for a recurrence, which means that the cancer has come back. Cancer recurs because small areas of cancer cells may remain undetected in the body. Over time, these cells may increase in number until they show up on test results or cause signs or symptoms.

During follow-up care, a doctor familiar with your child’s medical history can give you personalized information about the risk of recurrence. The doctor will ask specific questions about your child's health. Some people may have blood tests or imaging tests done as part of regular follow-up care, but testing recommendations depend on several factors, including the type and stage of cancer first diagnosed and the types of treatment given.

The anticipation before having a follow-up test or waiting for test results may add stress to you or a family member. This is sometimes called “scanxiety.” Learn more about how to cope with this type of stress.

Managing long-term and late side effects of Ewing sarcoma

Sometimes, side effects may linger beyond the active treatment period. These are called long-term side effects. In addition, other side effects called late effects may develop months or even years after treatment has ended. Late effects can occur almost anywhere in the body. They include physical problems, such as heart and lung problems, and second cancers, which is a new cancer that happens in someone who has had cancer before. People treated for Ewing sarcoma have a higher risk of developing leukemia and solid tumors. Late effects may also include emotional problems, such as anxiety and depression, and problems with memory, thinking, attention, and learning.

Based on the type of treatment the person with cancer received, the doctor will recommend what examinations and tests are needed to check for late effects. Children and young adults who received chemotherapy will need to be monitored for potential problems with their sexual health and development and fertility (ability to have children). Other late effects may include delayed or stunted body growth and delayed or missed developmental milestones. Your child may be referred to an endocrinologist, which is a doctor who specializes in problems with glands and the endocrine system, to identify and manage these late effects. Follow-up care should also address quality of life, including any developmental or emotional concerns.

The Children's Oncology Group (COG) has studied both the physical and psychological effects that cancer survivors from this age group face. Based on these studies, COG has created recommendations for long-term follow-up care for childhood, adolescent, and young adult cancer survivors that can be found on a separate website: www.survivorshipguidelines.org.

Keeping a child's personal health records

You are encouraged to organize and keep a personal record of your child’s medical information. The doctor will help you create this. That way, as your child enters adulthood, they have a clear, written history of the diagnosis, the treatments, and the doctor’s recommendations about the schedule for follow-up care. The American Society of Clinical Oncology (ASCO) offers forms to help keep track of the cancer treatment your child received and develop a survivorship care plan when treatment is completed.

Some children or young adults continue to see their oncologist, while others transition back to the care of their pediatrician, primary care doctor, or another health care professional. This decision depends on several factors, including the type and stage of cancer, treatments received, side effects, health insurance rules, and your family’s personal preferences. Talk with the health care team about your child’s ongoing medical care and any concerns you have about their future health.

If a doctor who was not directly involved in your child’s cancer care will lead the follow-up care, be sure to share the cancer treatment summary and survivorship care plan forms with them and with all future health care providers. Details about the specific cancer treatment given are very valuable to the health care professionals who will care for the person with cancer throughout their lifetime.

The next section in this guide is Survivorship. It describes how to cope with challenges in everyday life after a cancer diagnosis. Use the menu to choose a different section to read in this guide.

ON THIS PAGE: You will find some questions to ask your child's doctor or other members of the health care team to help you better understand your child's cancer diagnosis, treatment plan, and overall care. Use the menu to see other pages.

Talking often with the health care team is important to make informed decisions about your child's health care. These suggested questions are a starting point to help you learn more about your child's cancer care and treatment. You are also encouraged to ask additional questions that are important to your family. You may want to print this list and bring it to the next appointment or download Cancer.Net’s free mobile app for a digital list and other interactive tools to manage your child’s care. It may also be helpful to ask a family member or friend to come with you to appointments to help take notes.

Questions to ask after getting a diagnosis

• What type of Ewing tumor has been diagnosed?

• Where exactly is the tumor located?

• Can you explain the pathology report (laboratory test results) to me?

• What stage is the cancer? What does this mean?

Questions to ask about choosing a treatment and managing side effects

• What are the treatment options?

• What types of research are being done for Ewing sarcoma in clinical trials? Do clinical trials offer additional treatment options for my child?

• What treatment plan do you recommend? Why?

• What approach will be used for local control of the disease? Why?

• What is the goal of each treatment? Is it to eliminate the cancer, help my child feel better, or both?

• What are the possible side effects of each treatment, both in the short term and the long term?

• Who will be part of the health care team, and what does each member do?

• Who will be leading the overall treatment?

• Does this center specialize in the treatment of children and young adults with cancer?

• How will treatment affect my child's daily life? Will they be able to go to school and perform their usual activities?

• Could this treatment affect my child's ability to become pregnant or have children in the future? If so, should my family talk with a fertility specialist before treatment begins?

• If I’m worried about managing the costs of cancer care, who can help me?

• What support services are available to my family?

• If I have questions or problems, who should I call?

Questions to ask about having chemotherapy

• What chemotherapy will be given? How soon?

• What is the goal of this treatment?

• Where will the chemotherapy be given? At a hospital, clinic, or at home?

• How often will this treatment be given?

• How long will it take to give this treatment?

• What side effects can we expect during treatment?

• Who should I contact about any side effects my child experiences? And how soon?

• What are the possible long-term or late effects of having this treatment?

• What can be done to prevent or relieve the side effects?

• How can I best prepare for each treatment?

• Will other types of cancer treatments be needed?

Questions to ask about having surgery

• Will surgery be necessary?

• What type of surgery do you recommend? Why?

• How long will the operation take?

• If needed, what types of rehabilitative services are available?

• How long will my child need to stay in the hospital for?

• Can you describe what recovery from surgery will be like for my child?

• Who should I contact about any side effects my child experiences? And how soon?

• What are the possible long-term or late effects of having this surgery?

• What can be done to prevent or relieve the side effects?

Questions to ask about having radiation therapy

• Will radiation therapy be given?

• What is the goal of this treatment?

• How long will it take to give this treatment? How often will it be given?

• What side effects can we expect during treatment?

• Who should I contact about any side effects my child experiences? And how soon?

• What are the possible long-term or late effects of having this treatment?

• What can be done to prevent or relieve the side effects?

Questions to ask about planning follow-up care

• What is the chance that the cancer will come back? Should we watch for specific signs or symptoms?

• Is there anything my child can do to reduce their risk of getting another type of cancer?

• What long-term side effects or late effects are possible based on the cancer treatment given?

• What follow-up tests will my child need, and how often will those tests be needed?

• How do I get a treatment summary and survivorship care plan to keep in my child's personal records?

• When should my child return to their primary care doctor for regular medical care?

• Who will be leading the follow-up care?

• What survivorship support services are available to my family?

The next section in this guide is Additional Resources. It offers more resources on this website that may be helpful to you and your family. Use the menu to choose a different section to read in this guide.