Ewing Sarcoma

Ewing sarcoma refers to rare cancerous tumors in your bones and soft tissues like your muscles, tendons and ligaments. The condition, which is also known as Ewing tumor or Ewing’s sarcoma, may start in your hip, ribs or shoulder blades, thighbone or shinbone.

The tumors often develop during puberty, when your bones are growing very quickly. It typically affects people between ages 10 and 20, with most people receiving a diagnosis at age 15. But Ewing sarcoma may affect younger children and people in their late 20s to early 30s.

Types of this condition

There are three types of Ewing sarcoma:

• Ewing sarcoma of bone: This is the most common type.
• Extraosseous Ewing tumor (EOE): These tumors affect the soft tissues that surround your bones. Soft tissues include muscles, tendons and ligaments.
• Peripheral primitive neuroectodermal tumor (PPNET): This type of Ewing Sarcoma can start in bone or soft tissue.

Symptoms of Ewing sarcoma

Ewing sarcoma may look like a bump or lump on your arms, legs or chest. Other symptoms that you or your child could have include:

• Bone pain that comes and goes and seems to get worse at night
• Swelling in the tissue around bones. It may hurt when you touch your bones or the area next to them
• Lumps near the surface of your skin that may feel warm and soft to the touch
• Fever that doesn’t go away
• Broken bones that happen without an injury
• Fatigue and unexpected weight loss if Ewing sarcoma spreads

Ewing sarcoma causes

A genetic mutation (change) that happens after you’re born causes Ewing sarcoma. The change involves the EWSR1 gene and the FLI1 gene. The two genes fuse to create a new gene that causes cells to multiply and create cancerous tumors.

Researchers don’t know what triggers the change. But they’ve ruled out exposure to carcinogens that cause other types of cancer. That’s because Ewing sarcoma typically affects children and young adults who don’t experience long-term exposure to substances that can cause cancer.

Risk factors

A risk factor is something that can increase your risk of getting cancer or other diseases. The American Cancer Society lists the following risk factors for Ewing sarcoma:

• Your age: Anyone can get this disease, but it most often affects people ages 10 to 20.
• Your sex: Ewing sarcoma is slightly more common in males than females.
• Your race: The disease is more likely to affect people who are white (Hispanic or non-Hispanic) than people who are Asian American or Black.

Complications of Ewing sarcoma

Ewing sarcoma complications include cancer that spreads, comes back after treatment or a second cancer. A second cancer is a new and different cancer.

Your child may experience late effects. These are health issues that your child may have months or years after they complete treatment. Cancer and cancer treatment may lead to issues with your child’s:

• Organs and tissues
• Growth and development
• Moods, feelings and mental health
• Ability to think, learn and remember information
• Ability to have children

How doctors diagnose Ewing sarcoma

Healthcare providers will do a physical exam. They may feel for any lumps or bumps. They’ll ask about your symptoms. Tests to diagnose Ewing sarcoma may include:

• Imaging tests: Imaging tests let providers take a closer look at your bones and soft tissues. Common tests are CT scans, MRI scans, PET scans and bone scans.
• Biopsies: Providers get tissue samples for medical pathologists to examine under a microscope. They may do a needle biopsy or a bone marrow aspiration.
• Blood tests: These tests may show if cancer is spreading. Common blood tests are CBC with differential, CMP to measure enzymes and other substances, and LDH to measure enzyme levels.

How is Ewing sarcoma treated?

Chemotherapy is a common initial or first treatment, followed by surgery to remove the tumor. You may have more chemotherapy or radiation therapy after surgery. Other treatments are radiation therapy or surgery. An autologous stem cell transplant is a treatment option if the cancer isn’t in your bone marrow.

If your child has Ewing sarcoma, their provider may recommend that you consider enrolling them in a clinical trial. A clinical trial is research that studies new ways to treat diseases like Ewing sarcoma. Medical researchers test new treatments or combinations of existing treatments to see if the treatments are safe and effective.

A lot goes into the clinical trial process. You’ll probably have many questions about that process and how a trial may benefit your child. Take time to understand the benefits and potential risks. Your child’s cancer care team is a great resource if you’re considering this treatment option for your child.

When should I seek care?

Talk to your cancer care team if you or your child has:

• Treatment side effects that are more severe than you expect
• Pain that persists or gets worse even after taking prescription medication
• Symptoms that get worse or new symptoms

What is the survival rate for Ewing sarcoma?

Cancer survival rates vary depending on the cancer’s location at diagnosis. According to the American Cancer Society, Ewing sarcoma survival rates are:

• Localized tumors: 81%. This is cancer that hasn’t spread from where it started.
• Regional tumors: 77%, which means cancer is in nearby tissues.
• Distant tumors: 41%. This is cancer that’s spread from where it began to other bones, your lungs or bone marrow.

Survival rate data can be confusing and make you feel anxious. Try to keep in mind that survival rates are estimates that reflect others’ experiences. It’s natural to be concerned and have questions. Your child or your cancer care team understands all those feelings. They’ll take time to explain what survival rate data means in your or your child’s case.

How can I help my child?

Your child’s needs will change as they go through diagnosis, treatment and the rest of their life. For example, Ewing sarcoma typically happens around the time children start puberty. Puberty may feel like you and your teenager are on an emotional rollercoaster with sudden and unexpected twists and turns. A cancer diagnosis may put that emotional rollercoaster into overdrive.

Apart from feeling anxious about their health and coping with side effects, teenagers with Ewing sarcoma may feel frustrated about missing out on everyday activities at school and spending time with friends. They may also feel self-conscious about treatment side effects.

Try to be patient with your child and with yourself. Cancer can turn family life upside down, particularly if it’s cancer in your child. If you or your child feels overwhelmed, reach out to the child life specialist on your child’s care team. They’ll give you and your child helpful support and information.