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Ewing Sarcoma - Childhood - Overview

This section has been reviewed and approved by the Cancer.Net Editorial Board, 05/2014

ON THIS PAGE: You will find some basic information about this disease and the parts of the body it may affect. This is the first page of Cancer.Net’s Guide to the Ewing Sarcoma. To see other pages, use the menu on the side of your screen. Think of that menu as a roadmap to this full guide.

Ewing sarcoma is part of a group of cancers called the Ewing Family of Tumors (EFT) that affect the bones or nearby soft tissue. Cancer occurs when normal cells change and grow uncontrollably, forming a mass called a tumor. A tumor can be cancerous or benign. A cancerous tumor is malignant, meaning it can spread to other parts of the body. A benign tumor means the tumor will not spread.

Depending on the type, EFT develops in different places. EFT includes:

  • Ewing sarcoma: Also called Ewing tumor of the bones, this is the most common type of EFT.
  • Extraosseous Ewing (EOE): This is a tumor that grows in the soft tissues outside the bone.
  • Peripheral primitive neuroectodermal tumor (PPNET): This is a rare tumor found in the bones and soft tissues. This includes Askin's tumor, which is a PPNET of the chest wall.

Ewing sarcoma occurs most often in the leg, spine, rib, or pelvis. EFT of the soft tissue is usually found in the thigh, pelvis, spine, chest, or foot. Specifically, about 41% of EFT develops in a leg and foot, 26% in the pelvis, 16% in the chest wall, 9% in an arm or hand, 6% in the spine, and 2% in the skull.

This section covers Ewing sarcoma diagnosed in children and teenagers. For more information about Ewing sarcoma in adults, please read Cancer.Net's Guide to Bone Cancer.

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Ewing Sarcoma - Childhood - Statistics

This section has been reviewed and approved by the Cancer.Net Editorial Board, 05/2014

ON THIS PAGE: You will find information about how many children and teens learn they have this type of cancer each year and some general survival information. Remember, survival rates depend on several factors. To see other pages, use the menu on the side of your screen.

Each year, about 225 children and teenagers in the United States are diagnosed with Ewing sarcoma. Ewing sarcoma makes up about 1% of all childhood cancers. The five-year survival rate is the percentage of people who survive at least five years after the cancer is detected, excluding those who die from other diseases. The five-year survival rate of people with Ewing sarcoma with a localized tumor (a tumor that has not spread) is about 70%. If the tumor has metastasized (spread) at the time of diagnosis, the five-year survival rate is about 15% to 30%. The survival rate is slightly higher for patients whose tumor has spread to the lungs only, compared with those whose tumor has spread to the lungs and the bones.

Cancer survival statistics should be interpreted with caution. These estimates are based on data from a number of people with this type of cancer in the United States, but the actual risk for a particular individual may differ. It is not possible to tell a person how long he or she will live with Ewing sarcoma. Because the survival statistics are measured in five-year intervals, they may not represent advances made in the treatment or diagnosis of Ewing sarcoma. Learn more about understanding statistics.

Statistics adapted from the American Cancer Society.

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Ewing Sarcoma - Childhood - Risk Factors

This section has been reviewed and approved by the Cancer.Net Editorial Board, 05/2014

ON THIS PAGE: You will find out more about the factors that increase the chance of developing this type of cancer. To see other pages, use the menu on the side of your screen.

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 teens, 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 the father or mother. The genetic changes occur for no known reason. A high percentage of tumor cells have a chromosomal translocation, which means that small pieces of genetic material have swapped places inside the tumor cell. Usually the translocation is 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 uncontrolled growth of Ewing sarcoma cells.

Age. About two-thirds (64%) of all people with Ewing sarcoma are between the ages of 10 and 20. Ewing sarcoma is rare in adults older than 30 and in very young children.

Gender. Ewing sarcoma is more common among 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.

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Ewing Sarcoma - Childhood - Symptoms and Signs

This section has been reviewed and approved by the Cancer.Net Editorial Board, 05/2014

ON THIS PAGE: You will find out more about body changes and other things that can signal a problem that may need medical care. To see other pages, use the menu on the side of your screen.

Children and teens with Ewing sarcoma may experience the following symptoms or signs. Sometimes, a person with Ewing sarcoma does not show any of these symptoms. Or, these symptoms may be caused by a medical condition that is not cancer.

  • Stiffness, pain, swelling, or tenderness in the bone or in the tissue surrounding the bone. About 85% of children and teens with Ewing sarcoma have pain.
  • A lump near the surface of the skin that may feel warm and soft to the touch
  • A fever that doesn't go away
  • A broken bone that happens without an injury. A tumor growing in the bone can cause the bone to weaken or fracture (break).

If you are concerned about one or more of the symptoms or signs on this list, please talk with your child’s doctor. Your doctor will ask how long and how often your child has been experiencing the symptom(s), in addition to other questions. This is to help find out the cause of the problem, called a diagnosis.

If cancer is diagnosed, relieving symptoms remains an important part of your child’s cancer care and treatment. This may also be called symptom management, palliative care, or supportive care. Be sure to talk with your child’s health care team about symptoms he or she experiences, including any new symptoms or a change in symptoms.

The next section helps explain what tests and scans may be needed to learn more about the cause of the symptoms. Use the menu on the side of your screen to select Diagnosis, or you can select another section, to continue reading this guide.

Ewing Sarcoma - Childhood - Diagnosis

This section has been reviewed and approved by the Cancer.Net Editorial Board, 05/2014

ON THIS PAGE: You will find a list of the common tests, procedures, and scans that doctors can use to find out what’s wrong and identify the cause of the problem. To see other pages, use the menu on the side of your screen.

Doctors use many tests to diagnose Ewing sarcoma and find out if it has spread to another part of the body, called metastasis. Some tests may also determine which treatments may be the most effective. For most types of cancer, a biopsy is the only way to make a definitive diagnosis of cancer. If a biopsy is not possible, the doctor may suggest other tests that will help make a diagnosis. Imaging tests may be used to find out whether the tumor has spread. This list describes options for diagnosing this type of cancer, and not all tests listed will be used for every person. Your child’s doctor may consider these factors when choosing a diagnostic test:

  • Age and medical condition
  • Type of tumor suspected
  • Signs and symptoms
  • Previous test results

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

Blood tests. A complete blood count (CBC) is a blood test done to count 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 to determine the presence of a tumor in the body.

Imaging tests

X-ray. An x-ray is way to create 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.

Bone scan. A bone scan uses a radioactive tracer to look at the inside of the bones. 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 gray to the camera, and areas of injury, such as those caused by a tumor, appear dark.

Computed tomography (CT or CAT) scan. A CT scan creates a three-dimensional picture of the inside of the body with an x-ray machine. A computer then combines these images into a detailed, cross-sectional view that shows any abnormalities or tumors. A CT scan can also 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 to swallow.

Positron emission tomography (PET) scan. A PET scan is a way to create pictures of organs and tissues inside the body. 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. 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.

Magnetic resonance imaging (MRI). An MRI uses magnetic fields, not x-rays, to produce detailed images of the body. MRI can also 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 to swallow.

Surgical tests

Biopsy. A biopsy is the removal of a small amount of tissue 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.

During the procedure, the doctor may take a sample of the tumor itself; the soft, spongy tissue that is found in the center of larger bones, called bone marrow (see below); or both. Other tests can suggest that cancer is present, but only a biopsy can make a definite diagnosis. The sample removed during the biopsy is analyzed by a pathologist. 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 two procedures are similar and often done at the same time to examine the bone marrow. 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. The sample(s) are then analyzed by a pathologist. A common site for a bone marrow aspiration and biopsy is the hipbone in the lower back. The skin in that area is usually numbed with medication beforehand, and other types of anesthesia (medication to block the awareness of pain) may be used. 

Post-biopsy laboratory tests

Using the tissue sample removed during the biopsy, the doctor can conduct other laboratory tests to learn more about the tumor.

Immunohistochemistry. This test detects 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.

After diagnostic tests are done, your child’s doctor will review all of 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 helps explain the different stages for this type of cancer. Use the menu on the side of your screen to select Stages, or you can select another section, to continue reading this guide.

Ewing Sarcoma - Childhood - Stages

This section has been reviewed and approved by the Cancer.Net Editorial Board, 05/2014

ON THIS PAGE: You will learn about how doctors describe a cancer’s growth or spread. This is called the stage. To see other pages, use the menu on the side of your screen.

Staging is a way of describing where the cancer is located, if or where it has spread, and whether it is affecting other parts of the body. Doctors use diagnostic tests to find out the cancer’s stage, so staging may not be complete until all of the tests are finished. Knowing the stage helps the doctor to decide what kind of treatment is best and can help predict a patient’s prognosis, which is the chance of recovery. There are different stage descriptions for different types of cancer.

Although there is no official staging system for Ewing sarcoma, the following criteria help doctors describe Ewing sarcoma and work together to plan the best treatments:

Localized Ewing sarcoma. The tumor has remained in the bone or tissue where it began, or it has only spread to nearby tissues.

Metastatic Ewing sarcoma. The tumor has spread from the bone or tissue where it began to another part of the body, such as the lungs, other bones, or bone marrow. Rarely, the disease spreads to the lymph nodes, brain, or spinal cord. Approximately 25% of people with Ewing sarcoma will have obvious evidence of tumor spread at the time of diagnosis. Whether the tumor has spread is the most important factor used to determine a person’s treatment options and prognosis.

Recurrent Ewing sarcoma. Recurrent Ewing sarcoma is a tumor that has come back after treatment. It may recur where it began (a local recurrence) or in another part of the body (metastatic recurrence). If there is a recurrence, the cancer may need to be staged again (called re-staging).

Information about the cancer’s stage will help the doctor recommend a treatment plan. The next section helps explain the treatment options for this type of cancer. Use the menu on the side of your screen to select Treatment Options, or you can select another section, to continue reading this guide.

Ewing Sarcoma - Childhood - Treatment Options

This section has been reviewed and approved by the Cancer.Net Editorial Board, 05/2014

ON THIS PAGE: You will learn about the different ways doctors use to treat children and teens with Ewing sarcoma. To see other pages, use the menu on the side of your screen.

In general, cancer in children and teenagers is uncommon, so it can be hard for doctors to plan treatments unless they know what has been most effective in other children. That’s why more than 60% of children with cancer are treated as part of a clinical trial. Clinical trials are research studies that compare standard treatments (the best proven treatments available) with newer approaches to treatments that may be more effective. Clinical trials may test approaches such as a new drug, a new combination of standard treatments, or new doses of current therapies. Studying new treatments involves careful monitoring using scientific methods, and all participants are followed closely to track their health and progress.

To take advantage of these newer treatments, all children and adolescents 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. 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 their families, such as child life specialists, nutritionists, physical and occupational therapists, social workers, and counselors. Special activities and programs to help your child and family cope may also be available.

Children and teenagers with Ewing sarcoma should be treated in clinical trials specifically designed for their disease. A typical treatment plan for Ewing sarcoma consists of systemic therapy that treats the entire body, such as chemotherapy or stem cell transplantation, combined with localized therapy. Localized therapy focuses on treating the tumor itself using surgery and/or radiation therapy. When more than one treatment is used, it is called combination therapy.

Doctors make treatment decisions based on the stage of the disease and the age of the child, while trying to avoid or reduce long-term side effects of treatment. Descriptions of the most common treatment options for Ewing sarcoma are listed below. Your child’s care plan may also include treatment for symptoms and side effects, an important part of cancer care. Take time to learn about all of your child’s treatment options and be sure to ask questions about things that are unclear. Also, talk about the goals of each treatment with your child’s doctor and what your child can expect while receiving the treatment. Learn more about making treatment decisions.

Chemotherapy

Chemotherapy is the use of drugs to destroy cancer cells, usually by stopping the cancer cells’ ability to grow and divide. Chemotherapy is given by a pediatric or medical oncologist, a doctor who specializes in treating cancer with medication. Some children and teens may receive chemotherapy in their doctor’s office, while others may go to a hospital or outpatient clinic.

Systemic chemotherapy is delivered through the bloodstream to reach cancer cells throughout the body. Common ways to give chemotherapy include an intravenous (IV) tube placed into a vein using a needle or in 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. A chemotherapy regimen (schedule) usually consists of a specific number of cycles given over a set period of time. A patient may receive one drug at a time or combinations of different drugs at the same time.

When possible, chemotherapy is given before surgery. Sometimes, the doctor may use surgery or radiation therapy (see below) first and then give chemotherapy to eliminate any remaining Ewing sarcoma cells. After chemotherapy, the doctor may use localized surgery or radiation therapy followed by additional chemotherapy to eliminate any remaining Ewing sarcoma cells.

Patients with Ewing sarcoma may receive cyclophosphamide (Cytoxan, Neosar), doxorubicin (Adriamycin), ifosfamide (Ifex), etoposide (Toposar, VePesid), and vincristine (Oncovin, Vincasar PFS). For children and teens with Ewing sarcoma that has not spread to other parts of the body, the standard schedule is to receive chemotherapy every two 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 once treatment is finished.

Children and teens receiving chemotherapy for Ewing sarcoma may be at risk for developing neutropenia, an abnormally low level of a type of white blood cell called neutrophils. All white blood cells help the body fight infection. Your child’s doctor may give your child medications to increase his or her 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.

Learn more about chemotherapy and preparing for treatment. The medications used to treat Ewing sarcoma are continually being evaluated. Talking with your child's doctor is often the best way to learn about the medications prescribed for your child, their purpose, and their potential side effects or interactions with other medications. Learn more about your prescriptions by using searchable drug databases.

Surgery

Surgery is the removal of the tumor and surrounding tissue during an operation. When possible, surgical removal of the tumor should be performed after chemotherapy, called neoadjuvant chemotherapy. Surgery may also be needed to remove any remaining cancer cells after chemotherapy or radiation therapy. An orthopedic oncologist is usually the doctor who will perform the surgery. Learn more about cancer surgery.

Often a tumor can be removed without causing disability. However, if the tumor occurs in the 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 teens learn to use the limb again. Support services are available to help children and teens cope with the emotional effects of the loss of a limb. Learn more about rehabilitation.

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 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 (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 radiation therapy.

Stem cell transplantation/bone marrow transplantation

For Ewing sarcoma, stem cell transplantation is an experimental approach that is still under evaluation to determine its effectiveness in the treatment of this disease. It should only be done as part of a clinical trial.

A stem cell transplant is a medical procedure in which bone marrow that contains cancer is replaced by highly specialized cells, called hematopoietic stem cells, that develop into healthy bone marrow. Hematopoietic stem cells are blood-forming cells found both in the bloodstream and in the bone marrow. Today, this procedure is more commonly called a stem cell transplant, rather than bone marrow transplant, because it is the stem cells in the blood that are typically being transplanted, not the actual bone marrow tissue.

Before recommending transplantation, doctors will talk with the patient and family about the risks of this treatment and 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 two types of stem cell transplantation depending on the source of the replacement blood stem cell: allogeneic (ALLO) and autologous (AUTO). ALLO uses donated stem cells, while AUTO uses the patient’s own stem cells. AUTO transplantations are the type used to treat Ewing sarcoma.

In both types, the goal 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.

Learn more about stem cell and bone marrow transplantation.

Getting care for symptoms and side effects

Cancer and its treatment often cause side effects. In addition to treatment to slow, stop, or eliminate the cancer, an important part of cancer care is relieving a child’s symptoms and side effects. This approach is called palliative or supportive care, and it includes supporting the patient with his or her physical, emotional, and social needs.

Palliative care can help a person at any stage of illness. Children and teens often receive treatment for the cancer and treatment to ease side effects at the same time. In fact, patients who receive both often have less severe symptoms, better quality of life, and report they are more satisfied with treatment.

Palliative treatments vary widely and often include medication, nutritional changes, relaxation techniques, and other therapies. Your child may also receive palliative treatments similar to those meant to eliminate the cancer, such as chemotherapy, surgery, and radiation therapy. Talk with your child’s doctor about the goals of each treatment in the treatment plan.

Before treatment begins, talk with your child’s health care team about the possible side effects of the recommended treatment plan and supportive care options. And during and after treatment, be sure to tell your child’s doctor or another health care team member if your child experiences a problem so it is addressed as quickly as possible. Learn more about palliative care.   

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 “no evidence of disease” or NED.

A remission can be temporary or permanent. This uncertainty causes many children and their parents to feel worried or anxious that the cancer will come back. While many remissions are permanent, it is important to talk with your child’s 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 does return 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). Recurrence is most common within the first two years after treatment has finished. However, late recurrences occurring up to five years after treatment are more common with Ewing sarcoma than with other types of childhood cancers.

If there is a recurrence, a cycle of testing will begin again to learn as much as possible about the recurrence, including whether the cancer’s stage has changed. After testing is done, you, your child, and your child’s doctor will talk about the treatment options. The next round of treatment will depend on where and when the cancer recurred and how it was first treated. The doctor may use chemotherapy, including cyclophosphamide and irinotecan (Camptosar), temozolomide (Temodar, Methazolastone), and topotecan (Hycamtin), radiation therapy, and/or may surgically remove new tumors. Stem cell transplantation may also be recommended. Your child’s doctor may also suggest clinical trials that are studying new ways to treat this type of recurrent cancer.

Patients with recurrent Ewing sarcoma and their parents often experience emotions such as disbelief or fear. Patients and families are encouraged to talk with their health care team about these feelings and ask about support services to help them cope. Learn more about dealing with a recurrence.

If treatment fails

Although treatment is successful for the majority of children and teens with cancer, sometimes it is not. If a child’s cancer cannot be cured or controlled, this is called advanced or terminal disease. This diagnosis is stressful, and it may be difficult to discuss. However, it is important to have open and honest conversations with your child’s doctor and health care team to express your family’s feelings, preferences, and concerns. The health care team is there to help, and many team members have special skills, experience, and knowledge to support patients and their families.

Parents or guardians are encouraged to think about where the child would be most comfortable: at home, in a home-like setting elsewhere, in the hospital, or in a hospice environment. Hospice care is a type of palliative care for people who are expected to live less than six months. It is designed to provide the best possible quality of life for people who are near the end of life. Nursing care and special equipment can make staying at home a workable alternative for many families. Some children may be happier if they can arrange to attend school part-time or keep up other activities and social connections. The child’s health care team can help parents or guardians decide on an appropriate level of activity. Making sure a child 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.

The next section helps explain clinical trials, which are research studies. Use the menu on the side of your screen to select About Clinical Trials, or you can select another section, to continue reading this guide.

Ewing Sarcoma - Childhood - About Clinical Trials

This section has been reviewed and approved by the Cancer.Net Editorial Board, 05/2014

ON THIS PAGE: You will learn more about clinical trials, which are the main way that new medical approaches are tested to see how well they work. To see other pages, use the menu on the side of your screen.

Doctors and scientists are always looking for better ways to treat children and teens with Ewing sarcoma. To make scientific advances, doctors create research studies involving volunteers, called clinical trials.

Many clinical trials are focused on new treatments, evaluating whether a new treatment is safe, effective, and possibly better than the current (standard) treatment. These types of studies evaluate new drugs, different combinations of existing treatments, new approaches to radiation therapy or surgery, and new methods of treatment. Children and teens who participate in clinical trials are often among the first to receive new treatments before they are widely available. However, there is no guarantee that the new treatment will be safe, effective, or better than a standard treatment.

There are also clinical trials that study new ways to ease symptoms and side effects during treatment and managing the late effects that may occur after treatment. Talk with your child’s doctor about clinical trials regarding side effects.

Parents and children decide to participate in clinical trials for many reasons. For some, a clinical trial is the best treatment option available. Because standard treatments are not perfect, people are often willing to face the added uncertainty of a clinical trial in the hope of a better result. Other people volunteer for clinical trials because they know that these studies are the only way to make progress in treating Ewing sarcoma. Even if they do not benefit directly from the clinical trial, their participation may benefit future children with Ewing sarcoma.

Sometimes people have concerns that, by participating in a clinical trial, their child may receive no treatment by being given a placebo or a “sugar pill.” The use of placebos in cancer clinical trials is rare. When a placebo is used in a study, it is done with the full knowledge of the participants. Find out more about placebos in cancer clinical trials.

To join a clinical trial, parents and children must participate in a process known as informed consent. During informed consent, the doctor should list all of the child's options so that the parent and child understand the standard treatments and how the new treatment differs from the standard treatment. The doctor must also list all of the risks of the new treatment, which may or may not be different from the risks of standard treatment. Finally, the doctor must explain what will be required of each parent and child in order to participate in the clinical trial, including the number of doctor visits, tests, and the schedule of treatment.

For specific topics being studied for Ewing sarcoma, learn more in the Latest Research section.

People who participate in a clinical trial may stop participating at any time for any personal or medical reason. This may include that the new treatment is not working or there are serious side effects. Clinical trials are also closely monitored by experts who watch for any problems with each study. It is important that parents of patients participating in a clinical trial talk with their child’s doctor and researchers about who will be providing their treatment and care during the clinical trial, after the clinical trial ends, and/or if the patient chooses to leave the clinical trial before it ends. 

Cancer.Net offers a lot of information about cancer clinical trials in other areas of the website, including a complete section on clinical trials and places to search for clinical trials for a specific type of cancer.

The next section helps explain the areas of research going on today about this type of cancer. Use the menu on the side of your screen to select Latest Research, or you can select another section, to continue reading this guide.

Ewing Sarcoma - Childhood - Latest Research

This section has been reviewed and approved by the Cancer.Net Editorial Board, 05/2014

ON THIS PAGE: You will read about the scientific research being done now to learn more about this type of cancer and how to treat it. To see other pages, use the menu on the side of your screen.

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

New chemotherapy. Researchers are evaluating the use of cyclophosphamide, topotecan, and vincristine in addition to standard chemotherapy for patients with newly diagnosed, localized Ewing sarcoma.

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

Bilateral lung radiation. The effectiveness of bilateral lung radiation is being examined in patients with metastatic Ewing sarcoma. During this treatment, radiation therapy is given to both lungs after the completion of chemotherapy, and, for some patients, autologous (AUTO) stem cell transplant is recommended.

Insulin-like growth factor receptor-1 (IGF-1R) antibodies. The IGFR is an important growth protein for sarcomas. Blocking its activity may be an important new way to improve sarcoma treatment. This new type of treatment is being studied either by itself or in combination with chemotherapy for people with recurrent Ewing sarcoma.

Supportive care. Clinical trials are underway to find better ways of reducing symptoms and side effects of current Ewing sarcoma treatments in order to improve patients’ comfort and quality of life.

Looking for More About the Latest Research?

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

  • To find clinical trials specific to your diagnosis, talk with your child’s doctor or search online clinical trial databases now.
  • Visit ASCO’s CancerProgress.Net website to learn more about the historical pace of research for childhood cancer. Please note this link takes you to a separate ASCO website.

The next section addresses how to cope with the symptoms of the disease or the side effects of its treatment. Use the menu on the side of your screen to select Coping with Side Effects, or you can select another section, to continue reading this guide.

Ewing Sarcoma - Childhood - Coping with Side Effects

This section has been reviewed and approved by the Cancer.Net Editorial Board, 05/2014

ON THIS PAGE: You will find out more about steps to take to help your child cope with physical, social, and emotional side effects. This page includes several links outside of this guide to other sections of this website. To see other pages, use the menu on the side of your screen.

Fear of treatment side effects is common after a diagnosis of cancer, but it may help to know that preventing and controlling side effects is a major focus of your child’s health care team. This is called palliative or supportive care, and it is an important part of the overall treatment plan, regardless of the stage of disease.

Common side effects from each treatment option for Ewing sarcoma are described in detail within the Treatment Options section. Learn more about cancer and different treatments, along with ways to prevent or control them. Side effects depend on a variety of factors, including the cancer’s stage, the length and dosage of treatment(s), and your child’s overall health.

Before treatment begins, talk with your child’s doctor about possible side effects of each type of treatment your child will be receiving. Ask which side effects are most likely to happen, when they are likely to occur, and what can be done to prevent or relieve them. And, ask about the level of caregiving your child may need during treatment and recovery, as family members and friends play an important role in the care of children and teens with Ewing sarcoma. Learn more about caregiving.

In addition to physical side effects, there may be emotional and social effects as well. Patients and their families are encouraged to share their feelings with a member of the health care team who can help with coping strategies, including concerns about managing the cost of cancer care. These strategies can be different based on the age of your child or teen

During and after treatment, be sure to tell the health care team about the side effects your child experiences, even if you feel they are not serious. Sometimes, side effects can last beyond the treatment period, called a long-term side effect. A side effect that occurs months or years after treatment is called a late effect. Treatment of both types of effects is an important part of survivorship care. Learn more by reading the After Treatment section or talking with your child’s doctor.

The next section helps explain medical tests and check-ups needed after finishing cancer treatment. Use the menu on the side of your screen to select After Treatment, or you can select another section, to continue reading this guide.

Ewing Sarcoma - Childhood - After Treatment

This section has been reviewed and approved by the Cancer.Net Editorial Board, 05/2014

ON THIS PAGE: You will read about your child’s medical care after cancer treatment is finished and why this follow-up care is important. To see other pages, use the menu on the side of your screen.

After treatment for Ewing sarcoma ends, talk with your child’s doctor about developing a follow-up care plan. This plan may include regular physical examinations and/or medical tests to monitor your child’s recovery for the coming months and years. All children 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.

Children and teens who received chemotherapy will need to be monitored for potential problems with their sexual development and fertility (ability to have children). Your child may need to be referred to an endocrinologist, a doctor who specializes in problems with glands and the endocrine system, for evaluation in these areas. Other late effects may include delayed or stunted growth and delayed or missed developmental milestones. Learn more about the late effects of childhood cancer and find a Children’s Oncology Group follow-up clinic.

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 usually includes bone scans, CT scans, MRI scans, and x-rays. Your child’s doctor can recommend the necessary screening tests. Follow-up care should also address your child’s quality of life, including any developmental or emotional concerns. Learn more about childhood cancer survivorship.

Parents and guardians are encouraged to organize and keep a record of their child’s medical information. That way, as the child enters adulthood, he or she has a clear, written history of the diagnosis, the treatment given, and the doctor’s recommendations about the schedule for follow-up care. The doctor’s office can help you create this, and it should include recommendations from the doctor about the schedule for follow-up care. This information will be valuable to doctors who care for your child during his or her lifetime. ASCO offers cancer treatment summary forms to help keep track of the treatment your child received and develop a survivorship care plan once treatment is completed.

Children and teens who have had cancer can also enhance the quality of their future by following established guidelines for good health into and through adulthood, including not smoking, maintaining a healthy weight, eating a balanced diet, and participating in regular physical activity. Talk with the doctor about developing a plan that is best for your child’s needs. Learn more about the next steps to take in survivorship.

The next section offers a list of questions you may want to ask. Use the menu on the side of your screen to select Questions to Ask the Doctor, or you can select another section, to continue reading this guide.

Ewing Sarcoma - Childhood - Questions to Ask the Doctor

This section has been reviewed and approved by the Cancer.Net Editorial Board, 05/2014

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 diagnosis, treatment plan, and overall care. To see other pages, use the menu on the side of your screen.

Talking often with the doctor 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 you and your family. You may want to print this list and bring it to your child’s next appointment or download Cancer.Net’s free mobile app for an e-list and other interactive tools to manage your child’s care.

  • What type of EFT has been diagnosed?
  • Where exactly is the tumor located?
  • Can you explain the pathology report (laboratory test results) to me?
  • What are the treatment options?
  • What clinical trials are open to my child? Where are they located, and how do I find out more about them?
  • What treatment plan do you recommend? Why?
  • What is the goal of each treatment? Is it to eliminate the cancer, help my child feel better, or both?
  • What are the potential risks and benefits of each treatment?
  • Who will be part of the health care team, and what does each member do?
  • Who will be coordinating my child’s overall treatment and follow-up care?
  • Does this center specialize in the treatment of children, adolescents, and young adults with cancer?
  • What chemotherapy will my child receive? How often and for how long?
  • Will my child receive radiation therapy?
  • How can I best prepare my child for each treatment?
  • If needed, what types of rehabilitative services are available?
  • How long will my child need to stay in the hospital?
  • What are the possible side effects of this treatment plan, both in the short term and the long term?
  • How will treatment affect my child’s daily life? Will he or she be able to go to school and perform his or her 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 related to cancer care, who can help me with these concerns?
  • What follow-up tests will my child need, and how often will he or she need them?
  • What support services are available to my child? To the rest of my family?
  • Whom should I call for questions or problems?

The next section offers some more resources that may be helpful to you. Use the menu on the side of your screen to select Additional Resources, or you can select another section, to continue reading this guide.

Ewing Sarcoma - Childhood - Additional Resources

This section has been reviewed and approved by the Cancer.Net Editorial Board, 05/2014

ON THIS PAGE: You will find some helpful links to other areas of Cancer.Net that provide information about care and treatment for a person with cancer. This is the final page of Cancer.Net’s Guide to the Ewing Sarcoma. To go back and review other pages, use the menu on the side of your screen.

Cancer.Net includes many other sections about the medical and emotional aspects of being diagnosed with cancer, both for the child and their family members and friends. This website is meant to be a resource for you and your loved ones from the time of diagnosis, through treatment, and beyond. Here are a few sections that may get you started in exploring the rest of Cancer.Net:

This is the end of Cancer.Net’s Guide to the Ewing Sarcoma. Use the menu on the side of your screen to select another section, to continue reading this guide.