Ewing's Sarcoma

Overview

What is Ewing’s sarcoma?

Ewing sarcoma, also known as Ewing’s sarcoma and Ewing tumor, is a rare bone and soft tissue cancer that affects children and teenagers. Ewing sarcoma happens when the DNA in certain cells change and create abnormal cells that attack healthy tissue. Researchers are learning more about the connection between Ewing’s sarcoma and cell changes. That research is driving clinical trials of new Ewing sarcoma treatments. Children and young people with Ewing’s sarcoma might benefit by participating in such trials.

How common is Ewing’s sarcoma?

Each year about 200 children and teenagers are diagnosed with Ewing sarcoma. Ewing’s sarcoma represents about 1% of all cancers in children and adolescents under age 15, and 2% of all cancers in teenagers ages 15 to 19.

Who is affected by Ewing sarcoma?

Ewing’s sarcoma usually happens during puberty when young people’s bones are growing rapidly. More young males than females develop Ewing sarcoma.

How does this condition affect my child?

Ewing sarcoma is a bone and soft tissue cancer that typically appears in your child’s pelvis, ribs, shoulder blades and legs. Your child might complain that their bones hurt or they notice painful swollen areas on their body.

What is the survival rate for Ewing sarcoma?

Currently, 75% of children under age 15 who were treated for Ewing sarcoma are still alive five years after treatment, and 68% of young people ages 15 to 19 are still alive five years after treatment.

How aggressive is Ewing sarcoma?

Ewing sarcoma is an aggressive form of cancer that can spread to your children’s lungs, other bones and bone marrow.

What is the difference between Ewing sarcoma and osteosarcoma?

Osteosarcoma and Ewing sarcoma have different origins and come from different cells. This difference affects the treatment used. For example, radiation therapy is used more often to treat Ewing sarcoma than osteosarcoma.

Symptoms and Causes

What are the symptoms of Ewing’s sarcoma?

At first, Ewing’s sarcoma symptoms come and go before becoming consistent. About 85% of children and teenagers who Ewing sarcoma have pain. Other symptoms are:

  • Stiffness, swelling, or tenderness in the bone or in the tissue surrounding the bone.
  • Lumps near the skin’s surface that may feel warm and soft to the touch.
  • Fevers that don’t go away.
  • Broken bones that happen without an injury.
  • Weakness or numbness in the affected area.
  • Lack of energy.

What causes the condition?

Researchers believe Ewing sarcoma happens when certain cells change their DNA. Cells’ DNA tells them what to do. In the case of Ewing sarcoma, cells’ DNA tells healthy cells to ignore previous instructions that cause the cell to die. The healthy cells, now following their new instructions, go on to multiply and become a mass of abnormal cells. These abnormal cells attack healthy tissue, causing Ewing sarcoma.

Diagnosis and Tests

How is Ewing sarcoma diagnosed?

Your child’s healthcare provider will start with a physical examination and medical history. They might also use the following:

What tests are done to diagnose Ewing’s sarcoma?

  • Blood chemistry. This is a test on blood samples to see how well your child’s kidneys, liver and other organs are working. An abnormal amount of a substance in their blood can be a sign of disease or a side effect of treatment.
  • Biopsy.
  • Bone marrow aspiration.

Management and Treatment

How is Ewing sarcoma treated?

Ewing sarcoma treatment varies according to the specific cancer. Before beginning treatment, however, you might consider psychological support for your child, yourself and other family members. A Ewing sarcoma diagnosis can make you and/or your child to feel anxious, stressed and afraid. Obtaining counseling or help from a support group can help your family feel better prepared for treatment.

Treatment for Ewing sarcoma includes:

  • Chemotherapy.
  • Radiation therapy.
  • Surgery following radiation therapy to remove remaining cancer cells.
  • Targeted therapy. This treatment involves medications that target specific changes in cancer cells.

Prevention

Can Ewing sarcoma be prevented?

Unfortunately, researchers haven’t found specific lifestyle or environmental factors that might cause Ewing sarcoma.

Outlook / Prognosis

What can I expect after my child is treated for Ewing sarcoma?

Your child’s prognosis, or expected outcome, depends on several factors. Right now, 75% of children under age 15 who were treated for Ewing sarcoma are still alive five years after treatment, and 68% of teenagers ages 15 to 19 are still alive five years after treatment.

Your child will have a regular schedule of follow-up care so their healthcare provider can keep track of their recovery and watch for long-term side effects. Some children who had chemotherapy and radiation experience long-term side effects. Long-term side effects can develop months or years after treatment is done — they can be physical or emotional.

Living With

How can I take care of my child?

Managing treatment side effects is one of the challenging aspects of caring for a child who has Ewing sarcoma. Most cancer treatments have different side effects. Talk to your child’s healthcare provider about potential side effects. Ask what they will do to ease side effects and how you can help.

Your child might struggle with emotional side effects of cancer treatment. Studies show children, particularly adolescents, have a hard time with cancer treatment’s physical side effects such as gaining weight, losing hair or having scars from surgery. Talk to your provider about resources you can tap into to help your child work through their feelings.

When should my child see their healthcare provider?

Your child will have regular checkups during and after treatment. You should contact your provider if:

  • Your child has an unusually difficult time with treatment side effects. Your provider might recommend another approach.
  • Your child appears to develop new symptoms during or after treatment.

When does it make sense to go to the emergency room?

Generally speaking, 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 take your child to the emergency room if you’re concerned they are dehydrated.

What questions should I ask my doctor?

  • What type of Ewing tumor has been diagnosed?
  • What stage is the cancer? What does this mean?
  • Will surgery be necessary?
  • What type of surgery do you recommend and why?
  • What treatment plan do you recommend and why?
  • What is 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 the long term?
  • How will treatment affect my child's daily life? Will they be able to go to school and perform their usual activities?
  • What clinical trials are available? Where are they located, and how do I find out more about them?
  • What is 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 long-term side effects or late effects are possible based on the cancer treatment given?
  • What follow-up tests will my child need, and how often will those tests be needed?

A note from Cleveland Clinic

A cancer diagnosis is tough at any age. But it can be especially hard when it's Ewing sarcoma, which typically affects children and teenagers. Fortunately, many children and teenagers might qualify for clinical trials offering treatment tailored to Ewing sarcoma. Parents should talk to their healthcare providers about clinical trials. Parents should also ask about programs and resources to help their children who might be struggling with cancer’s social and emotional aspects.

Last reviewed by a Cleveland Clinic medical professional on 09/08/2021.

References

  • American Cancer Society. What’s New in Ewing Research and Treatment? (https://www.cancer.org/cancer/ewing-tumor/about/new-research.html) Accessed 9/29/2021.
  • American Society of Clinical Oncologists. Ewing Sarcoma – Childhood and Adolescence: Introduction. (https://www.cancer.net/cancer-types/ewing-sarcoma-childhood-and-adolescence/introduction) Accessed 9/29/2021.
  • American Psychological Association. Psychological Impact of Childhood Cancer. (https://www.apa.org/pi/families/resources/childhood-cancer-fact-sheet.pdf) Accessed 9/29/2021.
  • National Cancer Institute. Ewing Sarcoma Treatment. (https://www.cancer.gov/types/bone/patient/ewing-treatment-pdq#_280) Accessed 9/29/2021.
  • National Organization for Rare Disorders. Ewing Sarcoma. (https://rarediseases.org/rare-diseases/ewing-sarcoma/) Accessed 9/29/2021.

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