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Ewing Sarcoma - Childhood and Adolescence - Introduction

Approved by the Cancer.Net Editorial Board, 01/2019

ON THIS PAGE: You will find some basic information about Ewing sarcoma and the parts of the body it may affect. This is the first page of Cancer.Net’s Guide to Childhood Ewing Sarcoma. Use the menu to see other pages. Think of that menu as a roadmap for this complete guide.

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

Where Ewing tumors develop

Depending on the type, Ewing sarcomas develop in different places.

  • Bone. Ewing sarcoma most often develops in the leg, pelvis, rib, arm, or spine.

  • Soft tissue. Previously included in the term “Ewing sarcoma family of tumors,” Ewing sarcoma is a tumor that can grow in the soft tissue outside the bone. It is usually found in the thigh, pelvis, spine, chest wall, or foot.

Older terms, such as peripheral primitive neuroectodermal tumor, Askin tumor (Ewing sarcoma of chest wall), and extraosseous Ewing sarcoma (often combined in the term Ewing sarcoma family of tumors), refer to this same type of tumor.

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

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If you would like more of an introduction, explore these related items. Please note that these links will take you to other sections on Cancer.Net:

The next section in this guide is Statistics. It helps explain how many children and young adults are diagnosed with Ewing sarcoma, as well as general survival rates. Use the menu to choose a different section to read in this guide.

Ewing Sarcoma - Childhood and Adolescence - Statistics

Approved by the Cancer.Net Editorial Board, 01/2019

ON THIS PAGE: You will find information about the number of children and teens who are diagnosed with Ewing sarcoma each year. You will also read general information on surviving the disease. Remember, survival rates depend on several factors. Use the menu to see other pages.

Each year, about 200 children and teenagers in the United States are diagnosed with a Ewing tumor, most of which will be Ewing sarcoma. Ewing tumors make up about 1% of all childhood cancers. About half of all diagnoses of Ewing sarcoma are made in children between the ages 10 to 20. These tumors can also affect younger children and young adults in their 20s and 30s.

The 5-year survival rate tells you what percent of children and teens live at least 5 years after the cancer is found. Percent means how many out of 100. The 5-year survival rate is 78% for children under age 15 and 60% for adolescents aged 15 to 19.

Survival rates also depend on other factors, including how far the tumor as spread. The overall 5-year survival rate for people with a Ewing tumor that is only in the area it began (called localized) is 83%. If it has spread to the nearby region (called regionally), the 5-year survival rate is 69%. If the tumor has spread at the time of diagnosis, the 5-year survival rate is about 37%. There are other factors that have been linked to higher survival rates for all stages of Ewing tumors, such as: being under age 10, a smaller size tumor, if the tumor is in an arm or leg, normal levels of the enzyme lactate dehydrogenase (LDH) in the blood, and if chemotherapy is effective.

It is important to remember that statistics on the survival rates for children and teens with Ewing sarcoma are an estimate. The estimate comes from annual data based on the number of children and teens with this cancer in the United States. Also, experts measure the survival statistics every 5 years. So the estimate may not show the results of better diagnosis or treatment available for less than 5 years. Talk with your child’s or teen’s doctor if you have any have questions about this information. Learn more about understanding statistics.

Statistics adapted from the websites of American Cancer Society, National Cancer Institute, and St. Jude Children’s Research Hospital (January 2019).

The next section in this guide is Medical Illustrations. It offers drawings of body parts often affected by Ewing sarcoma. Use the menu to choose a different section to read in this guide.


Ewing Sarcoma - Childhood and Adolescence - Medical Illustrations

Approved by the Cancer.Net Editorial Board, 01/2019

ON THIS PAGE: You will find a basic drawing of the major bones in the body. Use the menu to see other pages.

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The illustration shows the major bones in the skeleton of an adolescent. Bones highlighted include the skull and bones in the torso such as the clavicle, ribs, sternum, vertebrae, and sacrum. It also highlights the bones of the arms, including the humerus, ulna, and radius and the bones of the legs, including the femur, tibia, and fibula.

Copyright 2013 American Society of Clinical Oncology (ASCO). Robert Morreale/Visual Explanations, LLC.

The next section in this guide is Risk Factors. It explains the factors that may increase the chance of developing Ewing sarcoma. Use the menu to choose another section to continue reading this guide.

Ewing Sarcoma - Childhood and Adolescence - Risk Factors

Approved by the Cancer.Net Editorial Board, 01/2019

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.

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. 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 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 years.

  • 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.

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

Ewing Sarcoma - Childhood and Adolescence - Symptoms and Signs

Approved by the Cancer.Net Editorial Board, 01/2019

ON THIS PAGE: You will find out more about body changes and other things that can signal a problem that may need medical care. Use the menu to see other pages.

Children and young adults with Ewing sarcoma may experience the following symptoms or signs. Sometimes, people with Ewing sarcoma do not have any of these changes. Or, the cause of a symptom may be a different 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 young adults with Ewing sarcoma have pain that can come and go and be less severe at night.

  • A lump near the surface of the skin that may feel warm and soft to the touch.

  • A fever that does not go away.

  • A broken bone that happens without an injury. A tumor growing in the bone can cause the bone to become weak or fracture.

If you are concerned about any changes you or your child experience, please talk with the doctor. The doctor will ask how long and how often the patient has been experiencing the symptom(s), in addition to other questions. This is to help figure out the cause of the problem, called a diagnosis.

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

The next section in this guide is Diagnosis. It explains what tests may be needed to learn more about the cause of the symptoms. Use the menu to choose a different section to read in this guide.

Ewing Sarcoma - Childhood and Adolescence - Diagnosis

Approved by the Cancer.Net Editorial Board, 01/2019

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 this happens, it is called metastasis. For example, imaging tests can show if the cancer has spread. Imaging tests show pictures of the inside of the body. 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.

This list describes options for diagnosing Ewing sarcoma. Not all tests listed below 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 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.

  • 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.

Imaging tests

  • 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 images 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 uses magnetic fields, not x-rays, to produce detailed images of the body. 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 relationship to nearby nerves and blood vessels.

  • Positron emission tomography (PET) or PET-CT scan. 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 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 or bone scan.

  • 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.

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, bone marrow (see below), or both. Other tests can suggest that cancer is present, but only a biopsy can make a definite diagnosis. A pathologist then 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 similar and often done at the same time to examine the bone marrow to 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.

    A pathologist then analyzes the sample(s). 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. Other types of medication may also be used to block the awareness of pain.

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 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, the 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 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.

Ewing Sarcoma - Childhood and Adolescence - Stages

Approved by the Cancer.Net Editorial Board, 01/2019

ON THIS PAGE: You will learn about how doctors describe a cancer’s growth or spread. This is called the stage. Use the menu to see other pages.

Staging is a way of describing where the tumor 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 is only found by physical examination or by imaging in the primary site or in lymph nodes next to the tumor. The tumor has not spread beyond that area.

  • Metastatic Ewing sarcoma. The tumor has spread from the primary site 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. Doctors will be able to quickly see evidence of tumor spread in 25% of children and young adults with Ewing sarcoma when they are diagnosed. 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. If the cancer does return, there will be another round of tests to learn about the extent of the recurrence. These tests and scans are often similar to those done at the time of the original diagnosis.

Information about the cancer’s stage will help the doctor recommend a specific treatment plan. The next section in this guide is Types of Treatment. Use the menu to choose a different section to read in this guide.

Ewing Sarcoma - Childhood and Adolescence - Treatment Options

Approved by the Cancer.Net Editorial Board, 01/2019

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. 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 or your child’s care.

Treatment overview

In many cases, a team of doctors works with the patient 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 and occupational therapists, social workers, and counselors. Special activities and programs to help your family cope may also be available.

It is important that patients are seen by medical experts from different specialties across disciplines who are experienced with treatment of Ewing sarcoma from the time of diagnosis. This includes radiologists, pediatric or medical oncologists, pathologists, surgical or orthopedic oncologists, and radiation oncologists. 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 a limb-sparing procedure will 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 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, radiation therapy, or both. When more than 1 treatment is used, it is called combination therapy.

Doctors make treatment decisions 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.

Descriptions of the common types of treatment used for Ewing sarcoma are listed below. The care plan may also include treatment for symptoms and side effects, an important part of cancer care.

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 you or 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 ending 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 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 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.

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, 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 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 the basics of chemotherapy.

Surgery

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

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.

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

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, 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.

Bone marrow transplantation/stem cell transplantation

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, 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. 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 bone marrow 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 transplants are used 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.

Care for symptoms and side effects

Cancer and its treatment often cause side effects. In addition to treatments intended to slow, stop, or eliminate the cancer, an important part of cancer care is relieving a patient’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 is any treatment that focuses on reducing symptoms, improving quality of life, and supporting patients and their families. Any person, regardless of age or type and stage of cancer, may receive palliative care. It works best when palliative care is started as early as needed in the cancer treatment process.

Children and young adults often receive treatment for the cancer at the same time that they receive treatment to ease side effects. In fact, people who receive both at the same time 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, emotional support, and other therapies. You or your child may also receive palliative treatments similar to those meant to eliminate the cancer, such as chemotherapy, surgery, or radiation therapy. Talk with the doctor about the goals of each treatment in the treatment plan.

Before treatment begins, talk with the health care team about the possible side effects of the recommended treatment plan and palliative care options. During and after treatment, be sure to tell the doctor or another health care team member if you or your child experiences a problem so it can be 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 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 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 2 years after treatment has finished. But 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 there is a recurrence, a new cycle of testing will begin again to learn as much as possible about the recurrence. 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 this type of recurrent cancer. Or he or she may suggest having the tumor analyzed for changes in genes that can be targeted by specific drugs. Whichever treatment plan you choose, palliative care will be important for relieving symptoms and side effects.

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

If treatment does not work

Although treatment is successful for the majority of 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 is there to help, and many team members have special skills, experience, and knowledge to support patients and their families.

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 patients may be happier if they can arrange to 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 young person 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 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.

Ewing Sarcoma - Childhood and Adolescence - About Clinical Trials

Approved by the Cancer.Net Editorial Board, 01/2019

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. Use the menu to see other pages.

What are clinical trials?

Doctors and scientists are always looking for better ways to care for children and young adults with Ewing sarcoma. To make scientific advances, doctors create research studies involving volunteers, called clinical trials. In fact, every drug that is now approved by the U.S. Food and Drug Administration (FDA) was tested in clinical trials.

Many clinical trials focus on new treatments. Researchers want to learn if a new treatment is safe, effective, and possibly better than the treatment doctors use now. These types of studies evaluate new drugs, different combinations of existing treatments, new approaches to radiation therapy or surgery, and new methods of treatment. People who participate in clinical trials can be some of the first to get a treatment before it is available to the public. But there are some risks with a clinical trial, including possible side effects and that the new treatment may not work. People are encouraged to talk with their health care team about the pros and cons of joining a specific study.

Some clinical trials study new ways to relieve symptoms and side effects during treatment. Others study ways to manage the late effects that may happen a long time after treatment. Talk with the doctor about clinical trials for symptoms and side effects. There are also clinical trials studying ways to prevent cancer.

Deciding to join a clinical trial

Families decide to participate in clinical trials for many reasons. For some people, 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 a way to contribute to the progress in treating Ewing sarcoma. Even if they do not benefit directly from the clinical trial, their participation may benefit future patients with Ewing sarcoma.

Insurance coverage of clinical trials costs differs by location and by study. In some programs, some of the expenses from participating in the clinical trial are reimbursed. In others, they are not. It is important to talk with the research team and your insurance company first to learn if and how the treatment in a clinical trial will be covered. Learn more about health insurance coverage of clinical trials.

Sometimes parents have concerns that, 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 in this way is rare overall and not done at all in childhood cancer research. Find out more about placebos in cancer clinical trials.

Patient safety and informed consent

To join a clinical trial, parents and their child must participate in a process known as informed consent. During informed consent, the doctor should:

  • Describe all of the treatment options so that the family understands how the new treatment differs from the standard treatment.

  • List all of the risks of the new treatment, which may or may not be different from the risks of standard treatment.

  • 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.

Clinical trials also have certain rules called “eligibility criteria” that help structure the research and keep patients safe. You and the research team will carefully review these criteria together.

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 children with cancer talk with the doctor and researchers about who will be providing their treatment and care during the clinical trial, after the clinical trial ends, and/or if they choose to leave the clinical trial before it ends.

Finding a clinical trial

Research through clinical trials is ongoing for all types of cancer. For specific topics being studied for Ewing sarcoma, learn more in the Latest Research section.

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.

In addition, this website offers free access to a video-based educational program about cancer clinical trials, located outside of this guide.

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The next section in this guide is Latest Research. It explains areas of scientific research currently going on for Ewing sarcoma. Use the menu to choose a different section to read in this guide.

Ewing Sarcoma - Childhood and Adolescence - Latest Research

Approved by the Cancer.Net Editorial Board, 01/2019

ON THIS PAGE: You will read about the scientific research being done now 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 you or your child.

  • Allogeneic 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, 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 is recommended but should only be done in a clinical trial.

  • Insulin-like growth factor receptor-1 (IGF-1R) antibodies. The IGF-1R is an important growth protein for sarcomas. Blocking its activity may be an effective new way to improve sarcoma treatment. This new type of treatment combined with chemotherapy is being studied for people newly diagnosed with metastatic Ewing sarcoma.

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

  • 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.

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:

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

Ewing Sarcoma - Childhood and Adolescence - Coping with Treatment

Approved by the Cancer.Net Editorial Board, 01/2019

ON THIS PAGE: You will learn more about coping with the physical, emotional, social, and financial effects of childhood cancer and its treatment. This page includes several links outside of this guide to other sections of this website. Use the menu to see other pages.

Every cancer treatment can cause side effects or changes to the patient's body and how he or she feels. For many reasons, people do not experience the same side effects even when they are given the same treatment for the same type of cancer. This can make it hard to predict how the person with cancer will feel during treatment.

As your family prepares to start cancer treatment, it is normal to fear treatment-related side effects. It may help to know that the health care team will work to prevent and relieve side effects. Doctors call this part of cancer treatment “palliative care” or "supportive care." It is an important part of the treatment plan, regardless of the person with cancer's age or the stage of disease.

Coping with physical side effects

Common physical side effects from each treatment option for Ewing sarcoma are described within the Types of Treatment section. Learn more about side effects of cancer and its treatment, along with ways to prevent or control them. Changes to the patient's physical health depend on several factors, including the cancer’s stage, the length and dose of treatment, and general health.

Sometimes, physical side effects can last after treatment ends. Doctors call these long-term side effects. They call side effects that occur months or years after treatment late effects. Treating long-term side effects and late effects is an important part of care for those diagnosed with cancer at an early age. Learn more by reading the Follow-up Care section of this guide or talking with the doctor.

Coping with emotional and social effects

Your family can have emotional and social effects as well as physical effects after a cancer diagnosis. This may include dealing with difficult emotions, such as anxiety or anger, or managing stress. Sometimes, patients and their families have problems expressing how they feel to their loved ones, or people don’t know what to say in response.

Patients and their families are encouraged to share their feelings with a member of their health care team. You can also find coping strategies for emotional and social effects in a separate section of this website. This section includes many resources for finding support and information to meet your family’s needs.

Coping with financial effects

Cancer treatment can be expensive. It is often a big source of stress and anxiety for families dealing with a cancer diagnosis. In addition to treatment costs, many people find they have extra, unplanned expenses related to their child’s care. Families are encouraged to talk about financial concerns with a member of their health care team. Learn more about managing financial considerations in a separate part of this website.

Caring for a child or teen with cancer

Family members and friends often play an important role in taking care of a person with Ewing sarcoma. This is called being a caregiver. As a parent or guardian, you are the primary caregiver for your child. But friends and family members can give your family valuable support, even if they live far away.

When a person has Ewing sarcoma, you may have an additional range of responsibilities. These may include giving medications or managing symptoms and side effects. However, it is important to seek help from others. Below are some of the responsibilities your family or friends could help with:

  • Providing short-term care for the person with cancer

  • Giving support and encouragement

  • Assisting with meals or household chores

  • Helping with insurance and billing issues

Learn more about caregiving.

Talking with the health care team about side effects

Before starting treatment, talk with the doctor about possible side effects. Ask:

  • Which side effects are most likely?

  • When are they likely to happen?

  • What can we do to prevent or relieve them?

Be sure to tell the health care team about any side effects that happen during treatment and afterward, too. Tell them even if you do not think the side effects are serious. This discussion should include physical, emotional, and social effects of cancer.

Also, ask how much care the person with cancer may need at home and with daily tasks during and after treatment. This can help you make a caregiving plan.

The next section in this guide is Follow-up Care. It explains the importance of checkups after cancer treatment is finished. Use the menu to choose a different section to read in this guide.

Ewing Sarcoma - Childhood and Adolescence - Follow-Up Care

Approved by the Cancer.Net Editorial Board, 01/2019

ON THIS PAGE: You will read about 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 to make sure the cancer has not returned, manage any side effects, and monitor the patient’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 or your child's recovery in the months and years ahead. Based on the type of treatment the patient 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. The doctor can recommend the necessary screening tests.

Learn more about the importance of follow-up care.

Watching for recurrence

One goal of follow-up care is to check for a recurrence. 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 or your child’s medical history can give you personalized information about the risk of recurrence. The doctor will ask specific questions about the patient’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 originally diagnosed and the types of treatment given.

The anticipation before having a follow-up test or waiting for test results can add stress to you or a family member. This is sometimes called “scan-xiety.” 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 afterwards. Late effects can occur almost anywhere in the body and include physical problems, such as heart and lung problems, as well as emotional and cognitive (memory, thinking, and attention) problems, such as anxiety, depression, and learning difficulties. Patients treated for Ewing sarcoma have a higher risk of developing later problems, such as leukemias and solid tumors, than people in the general population.

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 development and fertility (ability to have children). Other late effects may include delayed or stunted growth and delayed or missed developmental milestones. You or your child may be referred to an endocrinologist, 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 personal health records

You are encouraged to organize and keep a personal record of your or your child’s medical information. The doctor will help you create this. That way, as the patient 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. ASCO offers forms to help keep track of the cancer treatment you or 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 general care of their family doctor or another health care professional. This decision depends on several factors, including the type and stage of cancer, side effects, health insurance rules, and your family’s personal preferences. Talk with the health care team about your or your child’s ongoing medical care and any concerns you have about his or her future health.

If a doctor who was not directly involved in your or 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 him or her, 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 his or her 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.

Ewing Sarcoma - Childhood and Adolescence - Survivorship

Approved by the Cancer.Net Editorial Board, 01/2019

ON THIS PAGE: You will read about how to cope with challenges in everyday life after a cancer diagnosis. Use the menu to see other pages.

What is survivorship?

The word “survivorship” means different things to different people, but it often describes living with, through, and beyond cancer. In some ways, survivorship is one of the most complex aspects of the cancer experience because it is different for every patient and his or her family.

After active cancer treatment ends, patients and their families may experience a mixture of strong feelings, including joy, concern, relief, guilt, and fear. Some people say they appreciate life more after a cancer diagnosis. Other families stay very anxious about their child’s health and become uncertain about coping with everyday life.

One source of stress may occur when frequent visits to the health care team end after completing treatment. Often, relationships built with the cancer care team provide a sense of security during treatment, and survivors and their families miss this source of support. This may be especially true when new worries and challenges surface over time, such as any late effects of treatment, educational issues, emotional challenges, sexual development and fertility concerns, or financial issues.

Every family faces different concerns and challenges. With any challenge, a good first step is being able to recognize each fear and talk about it. Effective coping requires:

  • Understanding the challenge your family is facing

  • Thinking through solutions

  • Asking for and allowing the support of others

  • Feeling comfortable with the course of action your family chooses

It may be helpful to join an in-person support group or online community of childhood or adolescent cancer survivors. Support groups also exist for parents who have children diagnosed with cancer. This allows you to talk with people who have had similar first-hand experiences. Other options for finding support include talking with a friend or member of the health care team, individual counseling, or asking for assistance at the learning resource center of the place where the person with cancer received treatment.

Changing role of caregivers

Parents, other family members, and friends may also go through periods of transition. A caregiver plays a very important role in supporting a child or teen diagnosed with cancer, providing physical, emotional, and practical care on a daily or as-needed basis. Many caregivers become focused on providing this support, especially if the treatment period lasts for many months or longer.

However, as treatment is completed, the caregiver's role often changes. When the patient is a child or teenager, the need for caregiving related to the cancer diagnosis will become much less or come to an end as your child gets older. Family counselors at pediatric cancer centers can help with this transition. You can also learn more about adjusting to life after caregiving.

Healthy living after cancer

Survivorship often serves as a strong motivator to make positive lifestyle changes, often for the family as a whole.

Children and young adults who have had cancer can improve the quality of their future by following established guidelines for good health into and through adulthood. This includes not smoking, maintaining a healthy weight, eating well, getting enough sleep, managing stress, participating in regular physical activity, getting human papillomavirus (HPV) vaccinations, wearing seat belts when in a car or driving, and using sunscreen. Talk with the doctor about developing a plan that is best for the needs of the person with cancer. Learn more about making healthy lifestyle choices.

It is important that the person with cancer has recommended medical checkups and tests (see Follow-up Care) to take care of his or her health. Cancer rehabilitation may be recommended, and this could mean any of a wide range of services such as physical 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 and productive as possible.

Talk with the doctor to develop a survivorship care plan that is best for the survivor's needs.

Looking for More Survivorship Resources?

For more information about cancer survivorship, explore these related items. Please note these links will take you to other sections of Cancer.Net:

  • Survivorship Resources: Cancer.Net offers a lot of information and resources to help survivors cope, including specific sections for children, teens, and young adults. There is also a main section on survivorship for people of all ages.

  • ASCO Answers Cancer Survivorship Guide: Get this 44-page booklet that helps people transition into life after treatment. It includes blank treatment summary and survivorship care plan forms. The free booklet is available as a PDF, so it is easy to print out.

  • Cancer.Net Patient Education Video: View a short video led by an ASCO expert that provides information about childhood cancer survivorship.

The next section offers Questions to Ask the Health Care Team to help start conversations with the cancer care team. Use the menu to choose a different section to read in this guide.

Ewing Sarcoma - Childhood and Adolescence - Questions to Ask the Health Care Team

Approved by the Cancer.Net Editorial Board, 01/2019

ON THIS PAGE: You will find some questions to ask the doctor or other members of the health care team to help you better understand the 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 the health care. These suggested questions are a starting point to help you learn more about the 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 or your child’s care.

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?

  • 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?

Questions to ask about choosing a treatment and managing side effects

  • What are the treatment options?

  • What clinical trials are available? 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 the patient 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 the patient's daily life? Will he or she be able to go to school and perform his or her usual activities?

  • Could this treatment affect the person with cancer'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?

  • Whom should I call with questions or problems?

Questions to ask about having chemotherapy

  • What chemotherapy will be given?

  • What is the goal of this treatment?

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

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

  • What can be done to prevent or manage these side effects?

  • How can I best prepare for each treatment?

  • Will other treatments be needed?

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?

  • What side effects can we expect during treatment?

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

  • What can be done to prevent or manage these side effects?

Questions to ask about having surgery

  • How long will the patient need to stay in the hospital?

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

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

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 the person with cancer can do to reduce his or her 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 be necessary, and how often?

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

  • 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 some more resources on this website beyond this guide that may be helpful to you and your family. Use the menu to choose a different section to read in this guide.

Ewing Sarcoma - Childhood - Additional Resources

Approved by the Cancer.Net Editorial Board, 01/2019

ON THIS PAGE: You will find some helpful links to other areas of Cancer.Net that provide information about cancer care and treatment. This is the final page of Cancer.Net’s Guide to Ewing Sarcoma. Use the menu to go back and see other pages.

Cancer.Net includes many other sections about the medical and emotional aspects of being diagnosed with cancer, for the person diagnosed 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 links to help you explore other parts of Cancer.Net:

This is the end of Cancer.Net’s Guide to Ewing Sarcoma. Use the menu to choose a different section to read in this guide.