Ewing Sarcoma is a rare and aggressive Cancer that develops in the bones or, less commonly, in the soft tissues surrounding the bones, such as muscle or connective tissue. It most commonly affects children, teenagers, and young adults between the ages of 10 and 20. The average age of diagnosis is 15 years old.

There are over 400,000 children diagnosed with Childhood Cancer every year.

Ewing Sarcoma is named after Dr. James Ewing, an American pathologist who first described this bone Cancer in 1921. His discovery established Ewing Sarcoma as a distinct disease and helped to lay the foundation for modern research into its diagnosis and treatment. Dr. Ewing also played an important role in developing the institution that would later become Memorial Sloan Kettering Cancer Center.

More than 100 years later, treatment options for children whose Ewing Sarcoma returns remain extremely limited. This is why The Eli Sunshine Fund is dedicated to funding innovative research and cutting-edge clinical trials that give children more options and more hope.

Although anyone can develop Ewing Sarcoma, it most commonly affects:

• Children, teenagers, and young adults between the ages of 10 and 20

• Adolescents during periods of rapid growth

• Males slightly more often than females

• People of white or European ancestry more frequently than other racial or ethnic groups

Researchers do not believe Ewing Sarcoma is inherited, and there are currently no known lifestyle or environmental factors proven to cause the disease.

Symptoms vary depending on the size and location of the tumor.

The most common symptoms include:

• Persistent bone pain

• Swelling or tenderness

• A lump near the affected area

• Limping or difficulty walking

• Decreased range of motion

• Fatigue

• Fever

• Unexplained weight loss

If the tumor presses on nearby nerves, it may also cause numbness, tingling, weakness, or radiating pain.

Because Ewing Sarcoma is rare, its symptoms often resemble much more common conditions.

Children are frequently told they have:

• Sports injuries

• Growing pains

• Stress fractures

• Muscle strains

• Osteomyelitis

• As a result, diagnosis may take weeks or even months while other conditions are investigated first.

Persistent bone pain or swelling that continues to worsen despite treatment should always be evaluated by a medical professional.

Doctors use several tests to diagnose Ewing Sarcoma and determine whether it has spread.

Testing may include:

• X-rays

• MRI

• CT scan

• PET scan

• Blood work

• Bone marrow biopsy (when appropriate)

• Tumor biopsy

After the biopsy, specialized laboratory testing is performed to identify the characteristic genetic changes found in Ewing Sarcoma, most commonly an EWSR1 gene fusion, which helps confirm the diagnosis.

Treatment for Ewing Sarcoma is long and intensive.

Most patients receive 14 to 17 cycles of multi-agent chemotherapy over approximately 10 to 14 months. Chemotherapy is typically administered every two to three weeks and is combined with surgery, radiation therapy, or both, depending on each child’s individual treatment plan.

The standard chemotherapy regimen most commonly includes:

• Vincristine

• Doxorubicin

• Cyclophosphamide

• Ifosfamide

• Etoposide

Patients are treated by a multidisciplinary team that may include pediatric oncologists, orthopedic surgeons, radiation oncologists, pathologists, nurses, rehabilitation specialists, psychologists, social workers, child-life specialists, palliative care specialists, and supportive care providers.

Survival depends largely on whether the Cancer has spread before treatment begins.

• Localized Ewing Sarcoma: Approximately 70–80% five-year survival.

• Metastatic Ewing Sarcoma: Approximately 20–30% five-year survival.

Children whose Cancer has spread only to the lungs generally have better outcomes than those whose Cancer has spread to the bones or bone marrow.

Although many children respond well to their initial treatment, there are still very limited treatment options if Ewing Sarcoma returns.

Unlike many other Cancers, there is currently no universally accepted standard second-line treatment or “Plan B” for recurrent Ewing Sarcoma. Families are often faced with clinical trials, experimental therapies, or individualized treatment approaches because there is no established standard treatment once the disease recurs.

This is why research is so critical.

The Eli Sunshine Fund is committed to funding innovative research and cutting-edge clinical trials that give children more options, not only at diagnosis, but if their Cancer returns.

Every child deserves hope. Every family deserves more options. Every breakthrough brings us one step closer to a cure.