Can Ewing sarcoma be cured, and what research is underway?

Many patients with Ewing sarcoma are treated successfully, but survival has improved little over the past 30 years, and outcomes remain poor for patients whose disease has spread or returned, which is why continued research is urgently needed. Active areas of research include gene therapy, targeted drug therapy, and immunotherapy. The Ewing Sarcoma Institute accelerates collaborative research aimed at turning these approaches into better treatments and cures.

Resources for Patients & Families

Q: How is Ewing sarcoma diagnosed?

Ewing sarcoma is diagnosed with imaging studies such as X-rays, MRI, and CT scans, followed by a biopsy and genetic testing of the tumor tissue. Molecular testing identifies the defining EWSR1::ETS gene fusion, most commonly EWSR1::FLI1. Confirming the tumor's molecular signature is essential to an accurate diagnosis, and care is best carried out at a multidisciplinary center experienced with the disease.

Q: How rare is Ewing sarcoma?

Ewing sarcoma is rare, with approximately 200 children and teens diagnosed in the United States each year. It accounts for about 1% of all childhood cancers and is the second most common primary bone cancer in children and adolescents after osteosarcoma. Peak incidence occurs between ages 10 and 20, and the median age at diagnosis is 15.

A Ewing Sarcoma diagnosis can feel overwhelming, but understanding the disease and available treatments can help patients and families navigate the journey. Seeking expert care, staying informed, and connecting with support groups provide crucial guidance and support.

Sarcoma Treatment Centers

Ewing Sarcoma Treatment Centers

Patient & Family Resources

Because sarcomas are rare, it is important to find physicians and multidisciplinary treatment centers that have experience with this disease.

The Medical centers and hospitals specializing in sarcoma reflected on the map below meet the Sarcoma Foundation of America’s (SFA) criteria, which can be found here. If your institution meets this criteria and would like to be listed, please fill out the form on the SFA website.

For those seeking sarcoma centers outside the United States, Sarcoma Patient Advocacy Global Network (SPAGN) also provides a global directory of sarcoma centers to help patients identify specialized sarcoma care.

Search for a Clinical Trial

The Ewing Sarcoma Institute is pleased to connect interested parties with CareBox, a free and confidential service to search for clinical trials and that can guide you through a search and referral process to find a clinical trial that may be right for you. This search and matching system shortens the clinical trial search process from weeks to minutes, helping users identify clinical trials with eligibility criteria that match a patient’s specific diagnosis, stage, symptoms, and treatment history. Search the national database of Ewing Sarcoma trials and/or fill out your contact information below, and a Carebox Clinical Trial Navigator will call you within one business day. Se habla Español.

Dealing with Ewing Sarcoma

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Diagnosis

Treatment

Recovery

Frequently Asked Questions

What is Ewing Sarcoma?

Ewing sarcoma is a rare cancer that forms in bone or the soft tissue around bone, and it primarily affects children, adolescents, and young adults. It is the second most common primary bone cancer in young people after osteosarcoma.

Key characteristics:

Origin: Still under debate but most likely develops from primitive neuroectodermal or mesenchymal cells in bone or soft tissue
Age range: Most common between ages 10 and 20, though it can occur at any age
Genetic basis: Defined by a chromosome rearrangement, most often a translocation between chromosomes 11 and 22, that creates a characteristic gene fusion in the tumor cells between the EWSR1 gene and an ETS family gene (EWSR1::ETS), most often FLI1 leading to the EWS::FLI1 (EWSR1::FLI1) chimeric gene.
Common locations: Most often the long bones (femur, tibia), pelvis, ribs, and spine, but it can arise in any bone or soft tissue

What are the symptoms of Ewing sarcoma?

The most common symptoms of Ewing sarcoma are pain and swelling at the tumor site, often in a bone or the surrounding tissue. Because these symptoms can resemble a sports injury or growing pains, diagnosis is sometimes delayed.

Symptoms can include:

Pain and swelling at the tumor site
Bone pain that may worsen at night or with activity
Fever
Fatigue
Unexplained fractures caused by weakened bone

Anyone with persistent, unexplained bone pain or swelling should be evaluated by a physician.

How is Ewing Sarcoma diagnosed?

Ewing sarcoma is diagnosed with imaging studies followed by a biopsy and genetic testing of the tumor tissue. Confirming the tumor’s molecular signature is essential to an accurate diagnosis.

The process typically involves:

Imaging such as X-rays, MRI, and CT scans to locate and measure the tumor
A biopsy to confirm the presence of Ewing sarcoma cells
Genetic or molecular testing to identify the defining EWSR1::ETS gene fusion (most commonly EWSR1::FLI1)

Because Ewing sarcoma is rare, testing and care are best carried out at a multidisciplinary center experienced with the disease. A list of centers can be found on ESI’s website, under Patient & Family Resources.

How is Ewing sarcoma treated?

Ewing sarcoma is treated with a multidisciplinary combination of chemotherapy, surgery, and/or radiation therapy. The specific plan depends on the tumor’s location and size and whether the cancer has spread.

Treatment is tailored to each patient and generally coordinated by a team that includes medical oncology, surgical oncology, radiation oncology, and supportive care specialists. Seeking care at a center experienced with Ewing sarcoma is strongly recommended.

What are the side effects of Ewing sarcoma treatment?

Ewing sarcoma treatment can cause side effects including hair loss, nausea, fatigue, weakness, loss of appetite, and weight loss, most of which resolve after chemotherapy is completed. Surgery and rehabilitation carry their own considerations.

Common considerations by treatment type:

Chemotherapy: Hair loss, nausea, fatigue, weakness, reduced appetite, and weight loss, which typically resolve once treatment ends
Surgery: Often involves an internal prosthesis and, rarely, an external prosthesis
Recovery: Physical therapy during and after healing helps rebuild strength and movement safely

Is there a Ewing sarcoma cancer cluster in Ladera Ranch, California?

A number of Ewing sarcoma cases have been recently reported (7/26) among residents of Ladera Ranch, in Orange County, California, drawing significant media and community attention. The Ewing Sarcoma Institute’s role is to provide accurate scientific information and support to affected families. ESI does not determine whether a given group of cases constitutes a true cluster or what may have caused it, which are questions for public health authorities.

A cancer cluster is a greater-than-expected number of cases of the same cancer occurring within a defined group of people, in a specific geographic area, over a period of time. Because Ewing sarcoma is rare and its causes are not fully understood, several points are important for families and clinicians in Ladera Ranch and anywhere similar concerns arise:

The diagnosis has a specific molecular signature. Ewing sarcoma is defined by an EWSR1::ETS gene fusion, a genetic rearrangement that has never been reported in animals. Confirming this molecular diagnosis is essential in every case.
Every reported case should be molecularly confirmed. Establishing whether cases share the same diagnosis is the necessary first step before any pattern can be evaluated.
Confirmed cases can be studied in depth. Where diagnoses are confirmed, deeper genetic analysis (such as whole-genome sequencing and array CGH) can examine whether cases are related, and characterize the tumors’ mutational signatures.

Families in Ladera Ranch or elsewhere who are navigating a Ewing sarcoma diagnosis can reach the resources and clinical trial support on this page, and are encouraged to seek care at a multidisciplinary center experienced with the disease.

How rare is Ewing sarcoma?

Ewing sarcoma is rare, with approximately 250 children and teens diagnosed in the United States each year. It accounts for about 1% of all childhood cancers and is the second most common primary bone cancer in children and adolescents after osteosarcoma.

Peak incidence occurs between ages 10 and 20, and the median age at diagnosis is 15. It is slightly higher in males than in females. Incidence rates differ between ethnicities with highest incidence in people of European descent. Certain non-coding genomic variants at Ewing sarcoma susceptibility loci are more frequently found in affected individuals than in the general healthy population.

What causes Ewing Sarcoma?

Ewing sarcoma is caused by a genetic change in the tumor cells that is not inherited and is not passed from parent to child. In most cases the change is a fusion of the EWSR1 gene with an ETS family gene.

Specifically:

In roughly 85% of cases, a translocation between chromosomes 11 and 22 creates the EWS::FLI1 (EWSR1::FLI1) fusion gene
In the remaining cases, EWSR1 fuses with other ETS-family genes, most commonly ERG.
These changes arise spontaneously, most likely after birth, and are not inherited

There is no known lifestyle or environmental cause established for Ewing sarcoma.

Can Ewing sarcoma spread (metastasize)?

Yes. Ewing sarcoma can spread to other parts of the body, and its ability to metastasize is one of the most challenging aspects of treating the disease.

The most common sites of metastasis are:

Lungs (the most common site of distant spread)
Other bones beyond the original tumor
Bone marrow
Less commonly, lymph nodes and other organs

Whether the cancer has spread at diagnosis is a major factor in treatment planning and prognosis.

Are there clinical trials for Ewing sarcoma?

Yes. Clinical trials are available, particularly for patients whose disease returns after standard treatment, and they offer access to newer targeted therapies and immunotherapy combinations. Eligibility depends on each patient’s individual case.

What is the survival rate and prognosis for Ewing sarcoma?

The prognosis for Ewing sarcoma depends on several factors, including the tumor’s location and size, whether the cancer has spread, and the patient’s age and overall health. Patients with localized disease generally have better outcomes than those with metastatic disease.

Because prognosis varies so widely from person to person, survival statistics are best interpreted alongside a patient’s own care team.

A Guide for Patients and Families

For many patients and families, Ewing sarcoma is a disease they first learn about at the time of diagnosis. What was once an unfamiliar term quickly becomes central to their lives.

Understanding the disease is an important first step. Here, we explain what Ewing sarcoma is, how it presents, how physicians diagnose it and how it is treated today.

The Post-Diagnosis Ewing Sarcoma Guide

Navigate the path ahead with confidence—download our guide for essential information, resources, and support tailored for patients and parents.

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A Brief History of Ewing Sarcoma Treatment

Ewing Sarcoma treatment has evolved dramatically over the past century, transforming what was once an almost uniformly fatal disease into one with significantly improved survival rates.

Early Discovery and Initial Treatment Approaches (1920s-1950s)

James Ewing first described this aggressive bone tumor in 1921
Initially treated with radiation therapy alone
Survival rates were extremely poor, with most patients succumbing within months of diagnosis

Introduction of Chemotherapy (1960s-1970s)

Early single-agent chemotherapy showed modest benefits
The introduction of combination chemotherapy (particularly including cyclophosphamide, vincristine, doxorubicin, and actinomycin D) marked the first major breakthrough
Local control continued to rely heavily on radiation therapy

Multimodal Treatment Revolution (1980s-1990s)

Development of standardized protocols combining intensive chemotherapy, surgery, and radiation therapy
Introduction of neoadjuvant (pre-surgical) chemotherapy to shrink tumors before surgery
Improved surgical techniques allowed limb-sparing procedures instead of amputation in many cases
Five-year survival rates increased from under 10% to approximately 50-60%

Modern Era (2000s-Present)

Refinement of risk-adapted treatment approaches
Development of targeted therapies based on understanding of the EWS::FLI1 fusion protein and other molecular features
Improvements in radiation delivery techniques, including intensity-modulated radiation therapy
Enhanced supportive care to manage treatment toxicities
Current treatment typically involves 14-17 cycles of intensive multi-agent chemotherapy combined with local control measures (surgery and/or radiation)

Ongoing Clinical Trials & Breakthrough Research

We support and highlight cutting-edge clinical trials and research focused on advancing Ewing Sarcoma treatments. By accelerating innovation and expanding patient access, we aim to drive meaningful progress toward more effective and targeted therapies.