Ewing sarcoma

Here at the Dana-Farber/Children’s Hospital Cancer Center, we understand how overwhelming a diagnosis of Ewing sarcoma can be. We’ve provided some essential information in the following pages, and our experts will be sure to fully explain your child’s condition and recommended treatment plan when you meet with us.

Remember: we are always available to address any concerns and questions you have, at any point in the treatment process.

What is Ewing sarcoma?

• Ewing sarcoma is one of the Ewing family of tumors, named after the doctor who first described them in the 1920s.
• All of the tumors in the Ewing group grow from the same kind of stem cell.
• Ewing sarcoma is different from the other tumors in the Ewing family because it starts in either the bones or in the soft tissue that’s closest to the bones.
• Ewing sarcoma can occur in any bone, but most often is found in the pelvis, rib cage, spine, thigh or upper arm.
• Ewing sarcoma cells can spread (this is called metastasizing) to other areas of the body, including the lungs, bone and bone marrow and other soft tissues. This happens in about a third of all children with the disease.
• The cancerous cells can also spread to the kidneys, heart or adrenal gland, but this is extremely rare.

Who gets Ewing sarcoma?

• About 250 children and adolescents are diagnosed with the disease each year: Ewing sarcoma accounts for just 2 to 3 percent of all childhood cancers.
• Ewing sarcoma tends to affect children and young adults who are between 5 and 20 years old.
• The disease is extremely rare in children of African descent.
• Boys are more commonly affected than girls.

How serious is Ewing sarcoma?

• Ewing sarcoma is a potentially life-threatening cancer with a high risk of spreading to other parts of the body.
• Fortunately,treatments for Ewing sarcoma have improved dramatically in recent years.
• With early diagnosis and proper treatment, many kids who develop Ewing sarcoma have a good chance of recovery.

Causes

What causes Ewing sarcoma?

Ewing sarcoma occurs because a certain type of stem cell starts to grow abnormally, and these cells then form a tumor.

The exact cause of this abnormal cell growth isn’t fully understood. However, researchers have learned that chromosomal changes in a cell's DNA – the “building blocks” that make up all living organisms – can lead to Ewing sarcoma. These changes are not inherited; they develop in your child after he is born, and they happen for no apparent reason. It’s important to understand that there’s nothing you could have done or avoided doing that would have prevented your child’s cancer from developing.

Signs and symptoms

What are the symptoms of Ewing sarcoma?

While symptoms may vary from child to child, the most common include:  

• pain around the site of the tumor
• swelling and/or redness around the site of the tumor
• fever
• decreased appetite
• weight loss
• fatigue
• bowel or bladder incontinence (if the tumor is in the spinal region)
• symptoms related to nerve compression caused by the tumor (such as numbness, tingling or paralysis)

Because many of these symptoms can also point to other conditions, it’s important to have your child evaluated by a qualified medical professional right away.
 

Questions to ask your doctor

After your child is diagnosed with Ewing sarcoma, you may feel overwhelmed with information. It can be easy to lose track of the questions that occur to you.

Lots of parents find it helpful to jot down questions as they arise – that way, when you talk to your child’s doctors, you can be sure that all of your concerns are addressed. (If your child is old enough, you may want to suggest that she write down what she wants to ask her health care provider, too.)

Some of the questions you might want to start with include:

• How did you reach this diagnosis?
• Is there any other disease my child might have instead?
• Has my child’s tumor spread?
• Does he need to start chemotherapy? What about radiation?
• Can the tumor be treated with surgery?
• How long will my child need to be in the hospital?
• What are the possible short and long-term complications of treatment? How will they be addressed?
• What is the likelihood that my child will be completely cured?
• What happens if the tumor returns?
• How should I explain my child’s disease to him?
• How should I talk about his condition to family members and friends?
• What services are available to help my child and my family cope?
• Where can you point me for further information?

FAQ

Q: How are pediatric cancers different from adult cancers?

A: In general, childhood cancers are more successfully treated (meaning a larger percentage of patients are cured) compared with adult cancers. Why is this? Most likely, it’s because childhood cancers are usually more responsive to therapy, and a child can tolerate more intensive therapy when necessary. Read more about childhood cancers.

Q: Will my child be OK?

A: Today, many children with Ewing sarcoma can be cured. Even if the tumor comes back, further treatment approaches may be given successfully.

However, it’s important to understand that your child’s short-term health and long-term health can vary greatly depending on his specific circumstances. Prompt medical attention and intensive therapy are very important, as is continuous follow-up care.

If your child has Ewing sarcoma, his prognosis depends on:

• his age and overall health
• how far his disease has progressed at the time he’s diagnosed
• the size and location of the tumor(s)
• whether the tumor has metastasized(spread)
• how well his cancer responds to therapy
• his tolerance for specific medications, procedures or therapies

New methods for improving treatment and decreasing side effects are constantly being explored here at DF/CHCC. Learn more about our research into new treatments.

Q: What are the chances that Ewing sarcoma will occur in another child in our family?

A: Brothers and sisters of children with Ewing sarcoma are not at increased risk for developing this kind of cancer. That’s because children with Ewing sarcoma don’t inherit the disease from their parents (and they can’t pass on any increased risk of developing Ewing to their own children later in life, either).

Q: Will my child be better after she gets treatment?

A: Though your child’s prognosis will depend on her specific tumor and circumstances, with prompt and aggressive treatment, most children with Ewing sarcoma are successfully treated with a combination of chemotherapy and surgery and/or radiation. The majority of children have relatively few side effects from treatment.

Q: Will my child’s overall health be affected as she gets older?

A: As is the case with most cancers, a child with Ewing sarcoma may develop effects months or even years after treatment ends. These effects are called late effects, and may include:

• secondary cancers: Some chemotherapy drugs can cause leukemia. Alternatively, a new type of cancer can develop at the site of radiation therapy.
• fertility issues: All chemotherapy drugs carry fertility risks.

In addition, there is always the possibility of a recurrence of Ewing sarcoma.

Whether your child has any late effects, and the kind of effects she develops if she does have them, will depend on the location of her tumor and the specific treatment she received.

It’s important that you know what kind of treatment your child is getting and what impact these treatments might have on her life as she grows up. Ask your child's doctor to help you stay aware of possible long-term effects. And be sure your adolescent or adult child knows the details of her childhood cancer treatment so she can share that information with her doctors.

Q: What kind of long-term follow-up care should my child receive?

A: Some children who are treated for Ewing sarcoma and other pediatric cancers can experience significant long-term problems as a result of their treatment. All kids who have been treated for cancer require ongoing, specialized care to monitor for signs that their cancer may have returned, and to manage any side effects of treatment.

Here at Dana-Farber/Children’s Hospital Cancer Center, children usually return to us for care for several years after their initial cancer treatment. One of our major goals is to maximize your child’s long-term health. We do this in many ways, including assessing and attending to his:

• intellectual function and school performance
• endocrine functions
• neurological development
• mental and behavioral health
• hearing
• vision