Ewing Sarcoma

What is Ewing sarcoma?

Ewing sarcoma is a group of rare cancerous tumors that typically affect teenagers but can also affect younger kids and young adults. Ewing sarcoma, also known as Ewing’s sarcoma and Ewing tumor, usually affects people’s bones, but can also develop in nearby soft tissues. Thanks to ongoing research and newer treatments, more people with Ewing sarcoma are living longer cancer-free.

Types of Ewing sarcoma

There are three types of Ewing sarcoma:

• Ewing’s sarcoma of bone is the most common type of this condition.
• Extraosseous Ewing tumor (EOE) are tumors in soft tissues like muscles, tendons and ligaments that surround people’s bones.
• Peripheral primitive neuroectodermal tumor (PNET) also starts in bone or soft tissue but has a slightly different genetic makeup than Ewing sarcoma and EOE.

Is Ewing sarcoma common?

No, it’s not common. Each year in the U.S., between 200 and 250 people are diagnosed with Ewing sarcoma. People with Ewing sarcoma may have tumors in their arms and legs, pelvis or spine. The condition typically develops during puberty when young people’s bones are growing rapidly. Ewing sarcoma affects boys more often than it affects girls.

What are symptoms of Ewing sarcoma?

Ewing sarcoma symptoms can resemble common bumps or lumps and bruises on people’s arms, legs and chest. Parents and caregivers may mistake Ewing sarcoma symptoms for kids’ sports injuries. The difference is Ewing sarcoma symptoms go away but then come back, or they get worse. Common symptoms include:

• Bone pain that comes and goes and seems to get worse at night.
• Swelling in the tissue around bones. People may complain about feeling stiff or noticing it hurts when they touch some areas on their bones or next to their bones.
• Lumps near the skin’s surface 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 may be symptoms of metastatic Ewing sarcoma.

What causes Ewing sarcoma?

If your child has Ewing sarcoma, one of your first questions may be “Why is this happening?” While there’s no clear answer now, medical researchers know that a genetic mutation, or change, turns normal cells into Ewing sarcoma cells. These cells multiply and create cancerous tumors on people’s bones or soft tissues.

Researchers don’t know what triggers the mutation. But they do know the changes happen after birth. That means biological parents don’t pass the genetic mutation on to their biological children. Researchers also rule out exposure to carcinogens like tobacco, alcohol or chemicals that increase cancer risk.

What are Ewing sarcoma risk factors?

Risk factors are characteristics or activities that may increase the chance someone will develop a disease like cancer. Ewing sarcoma risk factors are things you can’t change or prevent:

• Age: People can develop Ewing sarcoma at any age, but more than 50% of people are diagnosed between ages 10 and 20. The median age at diagnosis is 15.
• Sex: Ewing sarcoma is more common in boys than in girls.
• Race/ethnicity: People who are white are more likely to develop Ewing sarcoma than people who are Black or Latino.

What are the complications of Ewing sarcoma?

Ewing sarcoma complications include:

• Metastatic cancer: Ewing sarcoma that spreads from the original tumor to other areas of your body is more difficult to treat.
• Recurrent cancer: Studies suggest recurrent Ewing sarcoma happens within the first two years after treatment, but it can come back as long as five years after treatment.
• Late effects: Late effects are medical conditions or issues linked to treatment that develop years after people complete treatment.

How is Ewing sarcoma diagnosed?

Healthcare providers will do a physical examination and ask questions about symptoms. They might also do the following tests:

Imaging tests

Imaging tests let providers take a closer look at your child’s bones and soft tissues and determine if there are tumors in other areas of their body. Imaging tests may include:

• X-rays.
• Magnetic resonance imaging (MRI).
• Computed tomography (CT) scan.
• Positron emission tomography (PET) scan.
• Bone scan.

Biopsies

Providers do biopsies to obtain tissue samples that medical pathologists examine under a microscope. Procedures may include:

• Needle biopsy: Providers use a thin hollow needle to obtain cells, fluid and tissue from suspicious lumps.
• Bone marrow aspiration: Providers look for signs of cancerous cells in liquid from the spongy material inside bone marrow.

Blood tests

Providers may do blood tests to determine if cancer has spread. Tests may include:

• Complete blood count (CBC) with differential: Low blood cell and platelet counts may be a sign of cancer.
• Comprehensive metabolic panel: This test measures the levels of enzymes and other substances in your blood.
• Lactate dehydrogenase (LDH): High LDH levels may be a sign cancer has damaged tissue.

How is Ewing sarcoma treated?

Chemotherapy is the most common initial treatment. Other treatments may include:

• Radiation therapy.
• Surgery following radiation therapy to remove remaining cancer cells.

Your child’s provider may recommend you consider enrolling your child in a clinical trial. In clinical trials, medical researchers test new treatments or combinations of existing treatments to determine if the treatments are safe and effective.

What are treatment side effects?

All cancer treatments cause side effects. The side effects vary depending on the treatment. For example, chemotherapy and radiation therapy side effects include:

• Diarrhea.
• Fatigue.
• Nausea and vomiting.

Regardless of treatment, people with Ewing sarcoma may want to consider palliative care. Palliative care helps ease symptoms and treatment side effects. Palliative care providers also help people understand treatment options.

What are treatment complications?

Kids who have Ewing sarcoma often have late effects. Late effects are health issues that surface months and years after diagnosis or treatment. These health issues may include second cancers, which are new and different cancers that develop long after they’ve completed cancer treatment. Cancer treatment may cause issues with people’s:

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

Can Ewing sarcoma be prevented?

There’s no known way to prevent Ewing sarcoma.

What can I expect if my child has Ewing sarcoma?

Your child’s prognosis, or expected outcome, depends on several factors, like:

• Your child’s age at diagnosis. Kids diagnosed before age 10 may have better prognoses than older kids and teenagers.
• Where the tumor started. Tumors that start in arms and legs are easier to treat than tumors in their spine and pelvis.
• The tumor’s size. Smaller tumors are easier to treat than larger tumors.
• Whether the tumor metastasized or spread by the time it was diagnosed.

What is the survival rate for Ewing sarcoma?

Ewing sarcoma survival rates vary depending on factors like a child’s age and whether cancerous tumors spread before diagnosis. Based on age, 78% of kids under age 15 and 68% of teenagers ages 15 to 19 were alive five years after diagnosis.

Survival rates based on tumor location at diagnosis are:

• An estimated 82% of people with localized tumors were alive five years after diagnosis. Localized tumors are tumors diagnosed before they spread from the area where they started.
• An estimated 71% of people with regional tumors were alive five years after diagnosis. Regional tumors include tumors that spread to nearby tissues before being diagnosed.
• An estimated 39% of people with metastatic tumors were alive five years after diagnosis. Ewing sarcoma tumors may spread to lungs, other bones or bone marrow.

It’s important to remember that Ewing sarcoma survival rates are estimates based on other people’s experiences with the condition. Their situations may be very different from yours. If you have questions, ask your healthcare provider to explain your or your child’s prognosis.

How do I take care of my child?

In a way, your child will be living with Ewing sarcoma for the rest of their life. That’s because late effects from cancer treatment can develop years after your child completes treatment. You can help your child by:

• Ensuring they have regular follow-up appointments after treatment.
• Encouraging them to develop good self-care habits, like having a healthy diet and getting exercise.
• Encouraging them to avoid activities, like smoking, that increase their risk of developing cancer again.

My child has Ewing sarcoma. What can I do to help them cope?

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 with friends. They may feel self-conscious about treatment side effects like losing hair. If that’s your family situation, talking with your child may help.

Cancer can turn family life upside down, particularly if it’s cancer in your child. If you or your child are feeling 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.

When should my child see their healthcare provider?

Ewing sarcoma can come back. Once your child completes treatment, they’ll continue to see their provider for follow-up tests, including X-rays, CT scans and bone scans so their provider can detect any recurring cancer as soon as possible.

When should I take my child to the emergency room?

You should go to the emergency room if your child has an unusually strong reaction to treatment. For example, some treatment side effects include nausea and diarrhea. You should also take your child to the emergency room if you’re concerned they’re dehydrated.

What questions should I ask my doctor?

You may feel overwhelmed when you first learn your child has cancer, in part because you don’t know what to expect at a time when you want to do everything you can to help your child. Here are some suggested questions that may help you find out what to expect and what you can do:

• What type of Ewing tumor does my child have?
• What stage is the cancer? What does this mean?
• What treatment plan do you recommend and why?
• What’s the goal of each treatment? Is it to eliminate the cancer, help my child feel better or both?
• What are the possible side effects of each treatment, both in the short term and long term?
• How will treatment affect my child’s daily life? Will they be able to go to school and perform their usual activities?
• Will surgery be necessary?
• What type of surgery do you recommend and why?
• What clinical trials are available? Where are they located, and how do I find out more about them?
• What’s the chance that the cancer will come back? Should I watch for specific signs or symptoms?
• Is there anything my child can do to reduce their risk of getting another type of cancer?
• What follow-up tests will my child need, and how often will they need tests?