Ewing sarcoma is cancer that can happen in your child’s bones and bone marrow. We don’t know what causes it, but we do know that it doesn’t run in families and is not caused by diet or lifestyle.
Almost all Ewing sarcoma patients need chemotherapy to shrink the primary tumor and prevent it from spreading to the lungs (metastasis). Your child may also need radiation therapy.
Ewing sarcoma is a malignant (cancerous) tumor that usually starts in bone but can also grow in soft tissue around a bone. It affects mostly teens and young adults ages 10 to 20. It is found most often in the longer bones of the arms and legs, but it can start in any bone, including the pelvis, ribs and spine. In about 1/3 of cases, it has already spread to the lungs, other bones or bone marrow by the time it is diagnosed. It occurs slightly more often in boys than girls. Ewing sarcoma does not seem to run in families, and it does not cause any symptoms before it is diagnosed.
A doctor who specializes in diagnosing and treating bone tumors, called a bone tumor specialist, will order tests to look for Ewing sarcoma. These may include X-rays, CT scans or MRI, blood work and a sample of the bone tissue for laboratory testing.
The lab test looks for changes in the DNA of the cancer cells. These are the same types of lab tests that doctors use to diagnose cancer in other parts of the body. In Ewing sarcoma, the chromosomes in tumor cells break apart and swap pieces with each other. This creates a new gene with altered activity that leads to cancer. In most cases, the changes in DNA that cause Ewing sarcoma happen for no apparent reason after birth.
Ewing sarcoma symptoms are pain, swelling or a lump in the area of the tumor. The tumor can cause the bone to bend or change shape, and it may feel warm or hard. It can also cause a fever and tiredness. Sometimes, cancer can spread from the bones to the lungs or other bones (metastasize).
Because the bone tumor specialist will want to know how far the Ewing sarcoma has spread, the specialist will do another type of test called a bone scan. The results of this test help the doctor plan treatment. For most patients, the first step is chemotherapy. This can be given in a vein, called intravenous (IV) therapy, or through an indwelling central venous catheter that stays in place until the end of treatment.
Ewing sarcoma is a type of bone cancer. It can cause pain, swelling and tenderness in and around a bone. It is usually found in and around the long bones of the arms, legs and pelvis. Children should see a doctor if they have signs or symptoms of this cancer. These include a lump in the arm, leg, chest or pelvis. A break in a bone or a swollen, painful area that won’t go away. If you think your child has a bone tumor, make an appointment with your family doctor or with a specialist called a pediatric oncologist.
Your child will need a variety of tests and procedures to diagnose this cancer. These tests will show if the cancer has spread (metastasized) and if it is high or low grade.
A biopsy is a procedure that removes a small piece of tissue from the tumor. A doctor can do a biopsy with a needle or with a surgical cut (incision). A doctor will then look at the biopsy sample under a microscope to check for cancer cells. Other tests may be done to find out if the cancer has spread. These may include blood tests, x-rays and an MRI.
If your child’s doctor thinks the tumor is Ewing sarcoma, they will order other tests to help them plan treatment. These may include a comprehensive metabolic panel, a test to measure the levels of certain enzymes in the blood, and a special blood test called lactate dehydrogenase.
These tests will help doctors find out how much cancer is in your child’s body and if it has spread. They will also do other imaging tests and a bone scan.
During treatment, your child will need strong medicines to kill cancer cells and stop them from growing. These are chemotherapy drugs. They may be taken by mouth or injected into a vein (IV). They are often given in cycles every two weeks. Your child might also need radiation therapy.
Sometimes, doctors will use a limb-sparing surgery to remove the tumor and a margin of healthy tissue. This is most often used in cases of Ewing sarcoma of the bones of the legs or pelvis.
Doctors use tests and procedures to diagnose Ewing sarcoma and find out how far the cancer has spread. This process is called staging. The doctors decide on the treatment plan based on the stage of the cancer.
Treatment may involve surgery, chemotherapy and/or radiation therapy. Sometimes doctors combine treatments. Chemotherapy is used first to help shrink the tumor, ease pain and kill additional cancer cells throughout the body. It is usually given through a vein (IV) in the arm or chest. The type of chemotherapy used depends on the location of the tumor and your child’s overall health. It can be given as pills, liquid or through a port placed surgically in the chest.
When the tumor is near a bone, doctors often use surgery to remove it along with some healthy tissue around it. This is called limb-sparing surgery. It is usually done under general anesthesia. If the cancer is near a joint, doctors will try to save the function of that joint by using reconstructive techniques.
Sometimes doctors need to give more chemotherapy after surgery to control the cancer and prevent it from coming back. They might also need to use radiation therapy or take more medicine to treat the rest of the body. Your child’s care team will discuss the best options for your child.
We follow up with Ewing sarcoma survivors to check for possible recurrence of the cancer and manage any side effects of treatment. This is very important because Ewing sarcoma has a tendency to recur, especially in younger people. We ask about any new signs or symptoms, how they started and how long they have lasted. We also ask about any medicines your child is taking, including vitamins and herbs.
Our cancer doctors are leaders in developing targeted therapies for childhood cancers. These agents are designed to attack the specific genes or proteins that drive the growth of cancerous cells. They are being tested in clinical trials to see if they can improve the way Ewing sarcoma is treated. These newer treatments are more likely to cause fewer side effects than traditional chemotherapy.
While relapse is common in Ewing sarcoma, advancements in local therapy and chemotherapy have improved survival. Patients with recurrent tumors may benefit from further local treatment or high-dose chemotherapy with stem cell rescue. Some new types of treatments are being tested in clinical trials. You can find NCI-supported cancer clinical trials that are accepting new patients by using the NCI Cancer Clinical Trials Search Tool.
Chemotherapy can cause serious side effects. Stem cells in the blood and bone marrow are damaged by high doses of chemotherapy. A procedure called stem cell transplant can replace these blood-forming cells after chemotherapy is done.
Ewing Sarcoma symptoms can be confusing. That’s why it is important to talk to your child’s doctor right away if he or she has any of the following symptoms.
Tests that create pictures of your child’s bones and soft tissue can help diagnose the condition and show whether it has spread. These tests include X-rays, magnetic resonance imaging (MRI), and bone scans.
Pain and swelling near a tumor are often the first symptoms of Ewing Sarcoma. The pain may get worse when the cancer grows. Sometimes, the tumor weakens a bone and causes it to break. This happens in one third of cases. Sometimes the cancer spreads to the lungs before doctors find it and treat it.
Doctors use several types of tests to diagnose Ewing Sarcoma and find out how far it has spread. These procedures make pictures of the bones and soft tissues in and around the area where the tumor is. They also find out if the cancer has spread to other parts of the body. This is called staging.
A physical exam is the first step in diagnosing Ewing Sarcoma. A doctor checks the area for lumps, redness and swelling. The doctor may ask questions about your child’s health history. This includes whether anyone in your child’s family has had cancer.
X-rays, magnetic resonance imaging (MRI), and computed tomography (CT) scans help doctors see the tumor better. These procedures also show if the cancer has spread to other bones or organs.
Ewing sarcoma usually occurs around the time of puberty. It can be hard for teenagers to deal with the emotional impact of a cancer diagnosis. They may also have trouble adjusting to treatment side effects.
Ewing sarcoma is caused by changes in DNA. Doctors don’t know what causes these changes. They do not understand why some kids are at higher risk for getting Ewing sarcoma than others. There is no link between the disease and diet, lifestyle or family history. Some children have a type of gene mutation that increases their chance of developing the disease. Doctors call this a “risk factor.” It is not the same as a disease caused by an infection or exposure to radiation or drugs.
When Ewing Sarcoma begins in a bone, it usually causes pain and swelling. The tumor also weakens the bone, so sometimes a minor injury may cause it to break. Children and young adults who have pain or a lump in a bone should see their doctor.
The doctor will get a detailed medical history and do a physical exam. Then the doctor will use tests to check for and diagnose Ewing Sarcoma. These include X-rays, magnetic resonance imaging (MRI), and a bone scan. The doctor may also order blood tests. These can show if cancer cells have spread from where they began to other parts of the body. This process is called staging.
Ewing Sarcoma mostly affects long bones, such as the thighbone (femur), shinbone (tibia), and arm bone (humerus). It sometimes starts in the soft tissues of the arms or legs. The tumor also can spread to the lungs.
To find out if a child has Ewing Sarcoma, the doctor will take a sample of the tissue around the tumor. This is done with a needle biopsy or during surgery. Then the doctor will do a bone marrow aspiration and biopsy. This is a procedure that takes fluid and tissue from the marrow inside the hip bone. The doctor will look for cancer cells in the bone marrow sample.
The doctor will use the results of these tests to decide on a treatment plan. Chemotherapy (medicine that kills cancer cells or keeps them from growing) is usually the first choice for Ewing Sarcoma. It is usually given for a long time. The chemotherapy drugs are often put into a vein (IV) in the chest. The doctor will put in an indwelling central venous catheter before starting chemotherapy so that the drugs can be delivered over a longer period of time.
Ewing sarcoma develops when cancer cells grow in the bone or soft tissue. It usually occurs in the pelvis, legs, arms or spine, and it affects more boys and people assigned male at birth (AMAB) than girls or those assigned female at birth (AFAB). It most often happens during puberty when young people’s bones are growing fast. It can also spread to the lungs and other bones (metastasize).
Children with Ewing sarcoma feel pain, swelling and tenderness at the site of the tumor. Sometimes the tumor weakens a bone and it breaks easily, especially after a minor injury. They may have a fever that doesn’t go away and feel very tired all the time. They also might lose weight.
Doctors treat Ewing sarcoma with surgery, chemotherapy and radiation. They can also use medicine to prevent the cancer from coming back after treatment. Doctors in many specialties help care for kids with this disease, including orthopaedic surgical oncologists, pediatric or adult medical oncologists, radiation oncologists and radiologists.
The first step in diagnosing Ewing sarcoma is to do a physical exam and take X-rays and CT scans of the area. They will also do a blood test called a complete blood count with differential, which measures the amounts of certain substances in the blood. A lower-than-normal amount of one substance, lactate dehydrogenase, can be a sign of cancer.
The next step is to get a biopsy, in which they remove some of the tumor and nearby tissue for examination under a microscope. This can be done with a needle or during surgery. They might also do a bone marrow aspiration and biopsy, in which they obtain liquid from the spongy material inside a bone and look for cancer cells.
A fever is a sign that cancer cells are active and may be spreading. If a child develops a fever, it is important for them to be seen by their doctor right away. A physician will perform a physical examination and take a blood sample. They may also need to do an X-ray or a CT scan. The doctor will ask the child about their symptoms and family history. They may refer them to a specialist hospital or bone tumour centre.
Ewing sarcoma is most common in teens and young adults, but it can affect people of any age. It often develops in the long bones of the arms and legs, chest, pelvis, or skull. Sometimes it forms in the soft tissue around the bones. It is less common for Ewing sarcoma to form in the flat bones of the trunk or the retroperitoneum (area in the back of the abdomen behind the abdominal wall).
Symptoms of a tumor can include pain and swelling near the site of the tumor. The doctor will use several types of tests to diagnose Ewing sarcoma and find out how far it has spread. These tests are called staging. They will look for cancer cells in the bones and soft tissue, and in other parts of the body.
A biopsy is a procedure that removes a small piece of the tumor to check for cancer cells. Other tests can include a chromosomal test to look for changes in the DNA that control how cells grow and die. The doctor can also measure the amount of certain proteins in the blood, such as lactic dehydrogenase, that are made by cancer cells.
A person with Ewing Sarcoma may lose weight as a result of the cancer and its treatment. This can lead to malnutrition, which can further affect a person’s overall health. The person’s healthcare team may prescribe nutritional supplements to help counteract this problem.
A medical professional can diagnose Ewing Sarcoma based on the symptoms and results of tests and procedures. These include imaging studies such as X-rays and CT scans, blood tests including comprehensive metabolic panel and lactic dehydrogenase (LDH), and an Ewing sarcoma biopsy. The biopsy involves taking a small sample of tissue from the tumor and examining it under a microscope to confirm the diagnosis and identify the type of sarcoma. It also helps determine the stage of the disease, which indicates whether it is localized only in one area or has spread to other parts of the body.
The genetic changes that cause Ewing sarcoma allow cancer cells to grow and divide faster than healthy cells, leading to the formation of a mass or tumor. These changes can also cause cancer cells to break off and spread to other areas of the body. When this happens, it is called metastatic cancer.
The treatment methods used to treat Ewing Sarcoma help prevent recurrence and improve survival. Two-thirds of patients who do not have their tumor spread will survive for at least 5 years after being diagnosed with standard treatment. However, some survivors experience health effects, known as late effects, months or years after their treatment is finished.
Survivors of Ewing sarcoma may need follow-up care for a long time to monitor any late side effects and check for any recurrence of the tumor. They should also get regular medical exams and have their bone and soft tissue examined by a doctor to look for any problems.
Your child’s care team will start with a physical exam. Then they will order tests to find out if the cancer has spread.
Chemotherapy uses drugs to kill cancer cells or stop them from growing. It is usually the first treatment. It might be given by mouth or injected into a vein (intravenously). It’s often combined with radiation therapy.
Ewing sarcoma is a type of bone cancer. It may spread (metastasize) to other parts of the body. It is usually diagnosed when doctors use tests to check for cancer in the bones and soft tissues. The tests include X-rays and CT or PET scans. Sometimes a biopsy of the tumor is done to confirm the diagnosis. A sample of the cancer cells is also tested to find out if the cancer has certain genetic changes.
Doctors give chemotherapy drugs to kill the cancer cells. They usually give this treatment before surgery to help decrease the chance that the cancer will come back. This is called neoadjuvant chemotherapy. Doctors also give high doses of chemotherapy after surgery to try to prevent the cancer from coming back in the area where it started. This is called adjuvant chemotherapy.
The most common chemotherapy treatments for Ewing sarcoma are doxorubicin (Adriamycin), vinblastine, and cyclophosphamide. These are often combined to make a more powerful chemotherapy drug.
A new type of chemotherapy treatment called tyrosine kinase inhibitors can also treat some types of Ewing sarcoma, especially those that have certain genetic changes. These drugs work by blocking the protein that cancer cells need to grow and divide.
There is no single test to diagnose Ewing sarcoma. Your child will need a complete physical exam, including X-rays and blood work. Then doctors will do other tests to find out if the cancer has spread.
If the cancer does not spread, treatment can be focused on removing the tumor and nearby tissues with surgery. If the cancer has already spread, more chemotherapy and radiation are needed. Other treatment options for recurrent Ewing sarcoma include a combination of drugs that target the cell’s ability to repair its DNA and a stem cell transplant. Researchers are looking into ways to improve current therapies for recurrent Ewing sarcoma, so we can cure more children, teens, and adults.
The first step in treating Ewing sarcoma is to find out how far the cancer has spread. Tests that make pictures of the bones and soft tissue help diagnose Ewing sarcoma and show whether it has spread. These tests include X-rays, CT scans, and MRI scans.
A doctor can also use a sample of tumor cells to look for specific changes in the DNA that cause Ewing sarcoma. These laboratory tests are called molecular tests. They can be done with a biopsy of a tumor or blood sample.
Surgery may be needed to remove a bone tumor, especially if it is large or in the backbone (spine). Radiation therapy may follow surgery to kill any remaining cancer cells. It may also be used before surgery to shrink the size of a large tumor and make it easier to remove.
If Ewing sarcoma has spread to the lungs, it is called metastatic disease. This is more serious and requires treatment that includes chemotherapy, radiation, and sometimes surgery.
All people with metastatic Ewing sarcoma should receive systemic combination chemotherapy. This is the standard of care for this type of cancer. This chemotherapy can help shrink the tumor before surgery or radiation and destroy any cancer cells that have spread to the lungs.
New types of treatment for Ewing sarcoma are being studied in clinical trials. These treatments may be better for some people with this type of cancer than standard therapy. To learn more about new treatments, talk to your child’s doctor. The doctor can check the cancer website for NCI-supported clinical trials that are accepting new patients and other sources of information about new treatments.
Radiation therapy uses X-rays and other types of radiation to kill cancer cells and shrink tumors. It may be given with chemotherapy, before surgery or by itself. Your child’s healthcare team will decide the best way to give this treatment.
If the bone cancer has not spread to other parts of the body, survival rates are very good. The chances of survival are even better if your child gets all of the treatment they need.
Your child’s doctor will do a physical exam and order other tests to diagnose Ewing Sarcoma. These might include an X-ray of the painful area, blood tests and an imaging test called a CT or MRI scan. Other tests might check how the bones are growing or whether the cancer has spread to the lungs or other bones. These tests might also be used to find out what type of ES your child has.
A biopsy is a procedure to take a small sample of tissue from the tumor for testing in a lab. The samples might be taken from the skin using a needle or through a small cut (incision) in the skin. These samples might also be checked to see what type of Ewing sarcoma your child has and which genes are involved. These are called cytogenetic tests.
There are new treatments for Ewing sarcoma that are being tested in clinical trials. These are often more effective than standard treatment and have fewer side effects. Your child’s healthcare team might be able to help you find a clinical trial for your child to join.
Researchers are looking for ways to treat Ewing Sarcoma by targeting specific changes in the cancer cells. They can use this information to find drugs that may help the tumor cells grow more slowly or die off completely.
The first step is to check for a bone tumor with a physical exam and some simple tests, like an X-ray or MRI scan. If the doctor thinks there may be a bone tumor, they’ll refer the person to a specialist called an orthopedic oncologist who deals with cancer that affects the bones.
This specialist might do a biopsy of the area, which involves taking a small piece of tissue from the affected bone (with a needle or during surgery) and testing it in a lab to see if it is cancer. They may also do a positron emission tomography scan, or PET, which takes pictures of the body’s cells and shows if there are any abnormal ones. Blood chemistry studies, which check for certain substances in the blood that are released by organs and tissues in the body, can also be done.
All of this information can help doctors choose the best treatment for a particular person. The goal is to treat the cancer and prevent it from returning. This is why people who have ES need follow-up care with their doctor for many years after treatment.
Recurrences of ES are usually very quick, and most people with recurrent ES have it within 2 years of their diagnosis. People who have recurrences of ES are more likely to get second cancers that affect the lungs and distant bones. They’re also at a higher risk of having other health problems, like heart and lung problems, emotional and learning issues and growth problems.
Stem cell transplant replaces the blood-forming cells (marrow) that were damaged by cancer or high doses of chemotherapy. It is used to treat cancers that affect the blood and bone marrow, such as leukemia, lymphoma and myelodysplastic syndromes. It is also being studied to treat other types of cancer.
To prepare for a stem cell transplant, doctors remove healthy blood-forming cells from the patient’s blood or bone marrow using a procedure called apheresis. This involves inserting a long, thin tube (catheter) into a large vein in the chest or neck. It is then connected to a machine that removes the stem cells and returns the rest of the blood. The stem cells are then frozen and stored.
Before the apheresis, the patient receives daily injections of growth factors that help increase the number of stem cells in the circulating blood and move them into the bone marrow. Doctors can then collect them. The donor’s blood is refilled with normal blood through another long, thin tube in the chest or neck (intravenous catheter). The catheter is left in place for the duration of treatment to deliver medications, nutrition and blood products.
The reinfused stem cells grow into normal, healthy blood cells and restore the body’s ability to fight disease. Patients with relapsed Ewing Sarcoma who receive high-dose chemotherapy and stem cell rescue have an overall survival of 60-70%. Sixteen of 31 patients who underwent transplant in a clinical trial using the ICE regimen (ifosfamide with mesna and cisplatin plus etoposide) showed an initial response (first complete response or partial response).
