Frequently Asked Questions About Ewing Sarcoma

Here are some answers to frequently asked questions about Ewing sarcoma.

Q: What is Ewing sarcoma?

A: Ewing sarcoma is a type of cancer. It can start in either the bone or in non-bony or soft tissues. Ewing sarcoma is part of the Ewing family of tumors (EFT). Ewing tumors that start in the soft tissue are called extraosseous or extraskeletal. Another member of the EFT family is the peripheral primitive neuroectodermal tumor (PPNET). These cancers are closely related. Experts believe they come from the same type of cells. They are treated in a similar way.

Q: Who gets Ewing sarcoma?

A: Most people who get Ewing sarcoma are between 10–20 years old. A small number of adults and younger children also get this cancer. More males get it than females. More white people get it than black people. Only about 1% of all childhood cancers are Ewing sarcoma. Experts aren’t sure why one person gets it and another doesn’t.

Q: What are the risk factors for Ewing sarcoma?

A: No lifestyle risk factors have been identified for Ewing sarcoma. Studies of children with this cancer have found no link between chemicals, radiation, or other exposure. Ewing sarcoma doesn’t seem to run in families. The only known risk factors are age, gender, and race. 

Q: What are the symptoms of Ewing sarcoma?

A: The symptoms of Ewing sarcoma depend on the person. They also depend on the size and location of the tumor. The most common symptom is pain in the area of the tumor. There may also be a lump, which may or may not hurt. The lump may be very painful if it’s near nerves or within the bone. Other symptoms may include fever, fatigue, and weight loss. These symptoms don’t mean that you have Ewing sarcoma. Other things could cause them. You should talk to your healthcare provider if you have any of these symptoms.

Q: How is Ewing sarcoma diagnosed?

A: Your healthcare provider will ask questions about your health history. He or she will do a physical exam to check for signs of cancer.

 The healthcare provider may order several tests to learn if there's cancer:

• X-rays

• Computed tomography (CT scan)

• Magnetic resonance imaging (MRI)

• Positron emission tomography (PET) scan

• Radionuclide bone scan

• Biopsy of the tumor

• Bone marrow aspiration and biopsy

Q: Should everyone get a second opinion for Ewing sarcoma?

A: Many people with cancer get a second opinion from another healthcare provider. There are many reasons to get one. These can include the following:

• Not feeling comfortable with the treatment decision

• Being diagnosed with a rare type of cancer

• Having several options for how to treat the cancer

• Not being able to see a cancer expert

Many people aren’t sure which Ewing sarcoma treatment would be best for them. It may help to have a second healthcare provider review the diagnosis and treatment options before starting treatment. In most cases, a short delay in treatment will not lower the chance that it will work. Some health insurance companies even require that a person with cancer seek a second opinion. They may even pay for a second opinion.

Q: How can someone get a second opinion for Ewing sarcoma?

A: These are some of the many ways to get a second opinion:

• Ask a primary care healthcare provider. He or she may be able to suggest a specialist. This may be a surgeon, medical oncologist, or radiation oncologist. Sometimes these healthcare providers work together at cancer centers or hospitals.

• Call the National Cancer Institute's Cancer Information Service. The number is 800-4-CANCER (800-422-6237). They have information about treatment facilities. These include cancer centers and other programs supported by the National Cancer Institute.

• Seek other options. Check with a local medical society or a nearby hospital or medical school. Or check with a support group to get names of healthcare providers who can give you a second opinion. Or ask other people who've had cancer for their suggestions.

Q: How is Ewing sarcoma treated?

A: Ewing sarcoma may be treated with surgery, radiation, or chemotherapy (chemo). A combination of these treatments is almost always used. Chemo tends to be the first treatment. If these treatments do not work, high-dose chemo followed by a stem cell transplant might be an option.

Q: What's new in Ewing sarcoma research?

A: Cancer research should give you hope. Healthcare providers and researchers around the world are learning more about what causes Ewing sarcoma. And they’re finding better ways to find and treat this cancer.

Scientists are working on new ways to diagnose the Ewing family of tumors. They’re using special proteins to look for substances in Ewing tumors that aren’t found in any other kind of tumor.

They’re also trying to understand the abnormal genes that cause Ewing sarcoma. Knowing more about these genes may help them form new treatments that turn off or inactivate the damaged genes.

Studies are testing new chemo medicines and high chemo doses to see how they’ll work on people with Ewing sarcoma.

Q: What should I know about clinical trials for Ewing sarcoma?

A: Clinical trials study new kinds of cancer treatments. Experts use clinical trials to learn how well new treatments work and what their side effects are. Promising treatments are ones that work better or have fewer side effects than the current treatments. People who participate in these studies sometimes get to use treatments before the U.S. Food and Drug Administration (FDA) approves them. People who join trials also help researchers learn more about cancer. They also help people who may get cancer in the future.