Ewing sarcoma

Date Updated: 01/07/2022

Overview

Ewing (YOO-ing) sarcoma is a rare type of cancer that occurs in bones or in the soft tissue around the bones.

Ewing sarcoma most often begins in the leg bones and in the pelvis, but it can occur in any bone. Less often, it starts in the soft tissues of the chest, abdomen, limbs or other locations.

Ewing sarcoma is more common in children and teenagers, but it can occur at any age.

Major advancements in the treatment of Ewing sarcoma have helped to improve the outlook for people with this cancer. After completion of treatment, lifelong monitoring is recommended to watch for potential late effects of intense chemotherapy and radiation.

Symptoms

Signs and symptoms of Ewing sarcoma include:

Pain, swelling or tenderness near the affected area
Bone pain
Unexplained tiredness
Fever with no known cause
Losing weight without trying

When to see a doctor

Make an appointment with your doctor if you or your child experiences any persistent signs and symptoms that worry you.

Causes

It's not clear what causes Ewing sarcoma.

Doctors know that Ewing sarcoma begins when a cell develops changes in its DNA. A cell's DNA contains the instructions that tell a cell what to do. The changes tell the cell to multiply quickly and to go on living when healthy cells would normally die. The result is a mass (tumor) of abnormal cells that can invade and destroy healthy body tissue. The abnormal cells can break away and spread (metastasize) throughout the body.

In Ewing sarcoma, the DNA changes most often affect a gene called EWSR1. If your doctor suspects that you have Ewing sarcoma, your cancer cells may be tested to look for changes in this gene.

Risk factors

Risk factors for Ewing sarcoma include:

Your age. Ewing sarcoma can occur at any age, but it is more likely to occur in children and teenagers.
Your ancestry. Ewing sarcoma is more common in people of European ancestry. It's much less common in people of African and East Asian ancestry.

Complications

Complications of Ewing sarcoma and its treatment include:

Cancer that spreads (metastasizes). Ewing sarcoma can spread from where it started to other areas, making treatment and recovery more difficult. Ewing sarcoma most often spreads to the lungs and to other bones.
Long-term treatment side effects. The aggressive treatments needed to control Ewing sarcoma can cause substantial side effects, both in the short and long term. Your health care team can help you manage the side effects that happen during treatment and provide you with a list of side effects to watch for in the years after treatment.

Diagnosis

Ewing sarcoma diagnosis usually begins with a physical exam to better understand the symptoms you or your child may be experiencing. Based on those findings other tests and procedures may be recommended.

Imaging tests

Imaging tests help your doctor investigate your bone symptoms, look for cancer and look for signs that the cancer has spread.

Imaging tests may include:

X-ray
Computerized tomography (CT)
Magnetic resonance imaging (MRI)
Positron emission tomography (PET)
Bone scan

Removing a sample of cells for testing (biopsy)

A biopsy procedure is used to collect a sample of suspicious cells for laboratory testing. Tests can show whether the cells are cancerous and what type of cancer you have.

Types of biopsy procedures used to diagnose Ewing sarcoma include:

Needle biopsy. The doctor inserts a thin needle through the skin and guides it into the tumor. The needle is used to remove small pieces of tissue from the tumor.
Surgical biopsy. The doctor makes an incision through the skin and removes either the entire tumor (excisional biopsy) or a portion of the tumor (incisional biopsy).

Determining the type of biopsy needed and the specifics of how it should be performed requires careful planning by the medical team. Doctors need to perform the biopsy in a way that won't interfere with future surgery to remove the cancer. For this reason, ask your doctor for a referral to a team of experts with extensive experience in treating Ewing sarcoma before the biopsy.

Testing the cancer cells for gene mutations

A sample of your cancer cells will be tested in the lab to determine which DNA changes are present in the cells. Ewing sarcoma cells usually have changes in the EWSR1 gene. Most often the EWSR1 gene becomes fused with another gene called FLI1, creating a new gene called EWS-FLI1. Testing the cancer cells for these gene changes can help confirm your diagnosis and give your doctor clues about the aggressiveness of your disease.

Treatment

Ewing sarcoma treatment usually begins with chemotherapy. Surgery to remove the cancer usually follows. Other treatments, including radiation therapy, might be used in certain situations.

Chemotherapy

Chemotherapy uses powerful drugs to kill cancer cells. Chemotherapy treatment usually combines two or more drugs that can be administered as an infusion into a vein (IV), in pill form, or through both methods.

Treatment for Ewing sarcoma usually begins with chemotherapy. The drugs may shrink the tumor and make it easier to remove the cancer with surgery or target with radiation therapy.

After surgery or radiation therapy, chemotherapy treatments might continue in order to kill any cancer cells that might remain.

For advanced cancer that spreads to other areas of the body, chemotherapy might help relieve pain and slow the growth of the cancer.

Surgery

The goal of surgery is to remove all of the cancer cells. But planning the operation also takes into consideration how it will affect your ability to go about your daily life.

Surgery for Ewing sarcoma may involve removing a small portion of bone or removing an entire limb. Whether surgeons can remove all of the cancer without removing the entire limb depends on several factors, such as the size and location of the tumor and whether it shrinks after chemotherapy.

Radiation therapy

Radiation therapy uses high-energy beams, such as X-rays and protons, to kill cancer cells.

During radiation therapy, the beams of energy are delivered from a machine that moves around you as you lie on a table. The beams are carefully directed to the area of the Ewing sarcoma in order to reduce the risk of damage to surrounding healthy cells.

Radiation therapy might be recommended after surgery to kill any cancer cells that remain. It can also be used instead of surgery if the Ewing sarcoma is located in a part of the body where surgery is not possible or would result in unacceptable functional outcomes (such as loss of bowel or bladder function).

For advanced Ewing sarcomas, radiation therapy can slow the growth of the cancer and help relieve pain.

Coping and support

A diagnosis of Ewing sarcoma can be frightening — especially for the family of a newly diagnosed child. With time you'll find ways to cope with the distress and uncertainty of cancer. Until then, you may find it helps to:

Learn enough about Ewing sarcoma to make decisions about care. Ask your doctor about Ewing sarcoma, including treatment options. As you learn more, you may become more confident in understanding and making decisions about treatment options. If your child has cancer, ask the health care team for guidance on sharing this information in a caring and age-appropriate way.
Keep friends and family close. Keeping your close relationships strong will help you deal with Ewing sarcoma. Friends and relatives can provide the practical support you'll need, such as helping take care of your home if your child is in the hospital. And they can serve as emotional support when you feel overwhelmed.
Ask about mental health support. The concern and understanding of a counselor, medical social worker, psychologist or other mental health professional also may help you. If your child has cancer, ask your health care team for advice on providing emotional and social support and options for professional mental health support. You can also check online for a cancer organization, such as the American Cancer Society, that lists support services.

Preparing for an appointment

If there are signs and symptoms that worry you, you'll likely start by making an appointment with your primary care doctor — or the pediatrician if the concern is with your child. If your doctor suspects Ewing sarcoma, make sure you're referred to an experienced specialist.

Ewing sarcoma typically needs to be treated by a team of specialists, which may include:

Orthopedic surgeons who specialize in operating on cancers that affect the bones (orthopedic oncologists)
Other surgeons, depending on the location of the tumor and patient age (for example, chest surgeons or pediatric surgeons)
Doctors who specialize in treating cancer with chemotherapy or other systemic medications (medical oncologists or, for children, pediatric oncologists)
Doctors who use radiation to treat cancer (radiation oncologists)
Doctors who analyze tissue to diagnose the specific type of cancer (pathologists)
Rehabilitation specialists who can help in recovery after surgery

What you can do

Before the appointment, make a list of:

Signs and symptoms, including any that seem unrelated to the reason for the appointment
Any medications being taken, including vitamins, herbs and over-the-counter medicines, and their dosages
Key personal information, including any major stresses or recent life changes

Also:

Bring previous scans or X-rays (both the images and the reports) and any other medical records related to this situation
Consider taking a relative or friend along to help you remember all the information provided during the appointment
Prepare a list of questions to ask the doctor to make the most of your time

Whether you're the patient or your child is the patient, your questions might include, for example:

What type of cancer is this?
Has the cancer spread?
Are more tests needed?
What are the treatment options?
What are the chances that treatment will cure this cancer?
What are the side effects and risks of each treatment option?
Will treatment affect the ability to have children? If so, do you offer fertility preservation evaluations and services?
Are there any brochures or other printed material that I can have? What websites do you recommend?

What to expect from your doctor

Your doctor will likely ask several questions. Be ready to answer them to allow more time to cover other points you want to address. Whether you're the patient or your child is the patient, the doctor may ask:

What are the signs and symptoms that you're concerned about?
When did you first notice these symptoms?
Have the symptoms been continuous or occasional?
How severe are the symptoms?
What, if anything, seems to improve the symptoms?
What, if anything, appears to worsen the symptoms?