Ewing Sarcoma

What is Ewing sarcoma?

Ewing sarcoma is a kind of cancer that grows in bones or soft tissues. The tumor was first described by a pathologist, Dr. James Ewing, in the 1920s. Most often, Ewing sarcoma is found in the bones of the pelvis or thigh, though it can arise throughout the body.

Most people with Ewing sarcoma have localized disease, meaning tests do not find signs of the tumor outside of the main tumor. About a quarter of people with Ewing sarcoma have metastatic disease, meaning tests show that the tumor has spread to parts of the body outside of the main tumor. When Ewing sarcoma spreads, it usually spreads to the lungs, other bones, or the bone marrow. Metastatic Ewing sarcoma is more difficult to treat.

Ewing sarcoma tends to strike children and young adults between the ages of 5 and 20, and is more common in boys than in girls. It is extremely rare in children of African descent. About 250 children and adolescents are diagnosed with Ewing sarcoma each year in the U.S., accounting for between 2 to 3 percent of all childhood cancers.

Maddie, an Ewing sarcoma patient at Boston Children

Meet Maddie

While enjoying college, this Ewing sarcoma survivor looks to her past.

What are the symptoms of Ewing sarcoma?

Ewing sarcoma symptoms can be non-specific and might mimic other more common ailments. While symptoms vary from child to child, the most common include:

  • sporadic bone pain of varying intensity
  • localized pain around the site of the tumor
  • limp or limited range of motion in the affected area
  • swelling and/or redness around the site of the tumor
  • fever of unknown cause
  • decreased appetite
  • weight loss
  • fatigue
  • symptoms related to nerve compression caused by the tumor (such as numbness, tingling, or weakness)
  • loss of bowel and bladder function (if the tumor is in the spinal region)

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

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. We understand now that a specific chromosomal change (called a “translocation”) in a cell's DNA — the building blocks that make up all living organisms — is one of the first events that turns a normal cell into a Ewing sarcoma cell. This translocation is not inherited but instead develops in cells after the child is born.

It is not known why some people are more likely to have cells that develop this translocation and therefore go on to develop Ewing sarcoma. It is important to understand that there’s nothing you could have done or avoided doing that would have prevented your child’s cancer from developing.

How is it classified?

The system used for staging Ewing sarcoma is simpler than the one used for most other cancers. Ewing sarcoma is classified as either localized or metastatic. Localized means the tumor has not spread beyond the initial location where it began or beyond the closest surrounding tissues. Metastatic means the tumor has spread to the lungs, bones, bone marrow, or to other organs or structures in the child’s body.

The classification (or stage), which is determined based on the results of imaging tests and biopsies, helps your doctors decide on treatment options and prognosis.

How we care for Ewing sarcoma

Fortunately, Ewing sarcoma treatments have improved dramatically in recent years, and most children who develop Ewing sarcoma have a good chance for recovery with proper treatment. Children diagnosed with Ewing sarcoma are treated at Dana-Farber/Boston Children's Cancer and Blood Disorders Center through our Bone and Soft Tissue Tumors Program. Our integrated pediatric oncology service offers — in one specialized program — the combined expertise of a leading cancer center and a world-renowned children’s hospital.

We provide the full set of options that can be used to treat bone and soft tissue tumors, and our specialists can help you determine which option is best for your child. Our pediatric oncologists have access to, and even lead, the most innovative clinical trials for Ewing sarcoma. Our surgeons have specialized expertise in complex surgeries, including limb-salvage surgery and rotationplasty, which may be used to treat Ewing sarcoma. And our radiation oncologists have access to the newest radiation therapy techniques that may also be used in the care of a child with Ewing sarcoma.

Our areas of research for Ewing sarcoma

Dana-Farber/Boston Children’s is one of the top pediatric research centers in the world, with top researchers in Ewing sarcoma. Our research program includes laboratory scientists and clinical researchers from both Dana-Farber Cancer Institute and Boston Children’s Hospital. We investigate Ewing sarcoma from every angle — from examining cells under the microscope to tracking response to current drug regimens — so that we can create better treatments for your child.

Our laboratory researchers have helped to define the gene changes in Ewing sarcoma cells beyond the known translocation seen in these cells. We are also developing tools to detect low levels of Ewing sarcoma cells in the blood and bone marrow. These tools may help us to improve how we monitor response to treatment.

Ewing sarcoma clinical trials

Clinical trials, or research studies evaluating new treatment approaches, are a major offering at Dana-Farber/Boston Children’s. We were one of the first centers to perform limb-salvage surgery for patients with bone cancers like Ewing sarcoma. Today, our clinical researchers continue to develop new approaches to the treatment of Ewing sarcoma.

We are now conducting numerous clinical trials, mainly focusing on patients with the hardest-to-treat forms of Ewing sarcoma: metastatic Ewing sarcoma and Ewing sarcoma that has come back after initial treatment (called “relapsed” or “recurrent” disease). For example, we are leading a clinical trial for children and young adults with newly diagnosed metastatic Ewing sarcoma that compares standard treatment to standard treatment with the addition of a drug that blocks an important growth pathway in Ewing sarcoma (called the “IGF-1R pathway”).

We offer enrollment in both independent and cooperative treatment studies, many of which are available only at our center. If you’re not sure which clinical trials might be right for your child, email us at clinicaltrials@danafarberbostonchildrens.org. We can help you navigate your options. The Children's Oncology Group is a consortium of cancer treatment centers across the United States, Canada, and other countries that conduct studies of nearly every kind of pediatric cancer. Our participation in this group gives children with cancer unparalleled access to the newest clinical trials.