Ewing Sarcoma

Ewing sarcoma is a cancer of the bone or the soft tissue around bones. It most often affects teenagers and young adults, usually in the pelvis, thigh bone, shin bone, upper arm, ribs or chest wall.

Symptoms

• Pain in a bone, often worse at night, and swelling or a lump
• A bone that breaks after a minor injury
• Fever, tiredness and weight loss
• Breathing problems if the tumor is in the chest

Causes

Ewing sarcoma is caused by an acquired gene change in the tumor cells — usually a fusion between the EWSR1 and FLI1 genes. It is not inherited.

Risk factors

• Age 10–20 years
• Slightly more common in boys
• Much more common in people of European ancestry

Prognosis and survival

For localized Ewing sarcoma, about 70–80% of patients are cured with combined treatment. Outcomes are lower when the tumor has spread at diagnosis, but many patients still achieve long-term control.

Diagnosis confirms Ewing sarcoma and checks whether it has spread, which decides the treatment plan.

Complete blood count (CBC)

Along with LDH, which is often raised when the disease is more extensive.

Imaging tests

• X-ray — the first test for bone pain
• MRI — shows the full extent of the tumor in bone and soft tissue
• CT of the chest — looks for spread to the lungs
• PET-CT and bone scan — look for spread elsewhere

Biopsy

A needle or surgical biopsy, ideally performed at the center that will do the final surgery, so that the biopsy track can be removed later.

Bone marrow aspiration and biopsy

Checks for spread to the marrow.

Immunohistochemistry, chromosome tests and FISH

Confirm the diagnosis by showing CD99 on the cells and the EWSR1 gene rearrangement.

Staging

Ewing sarcoma is described as localized (only at the primary site and nearby tissue) or metastatic (spread to lungs, bones or bone marrow).

Treatment combines chemotherapy with local control of the tumor by surgery, radiation or both.

Your child's treatment plan depends on:

• Where the tumor is and how large it is
• Whether it has spread
• How well it responds to the first chemotherapy
• Age and general health

Chemotherapy

Given for several months before and after local treatment, to shrink the tumor and destroy hidden cancer cells.

Surgery

Removes the tumor with a margin of healthy tissue. Limb-sparing surgery with implants or bone grafts is used whenever possible.

Radiation therapy

Used instead of surgery when the tumor cannot be removed safely, or after surgery if margins are close. Lung radiation may be given for lung metastases.

Bone marrow / stem cell transplant

High-dose chemotherapy with an autologous stem cell transplant may benefit selected patients with high-risk or relapsed disease.

Targeted therapy

New targeted treatments are available in clinical trials for relapsed disease.

Our sarcoma team — oncologists, orthopedic surgeons and radiation oncologists — plan every case together.