Ewing Sarcoma

Diagnosis

Diagnosing Ewing sarcoma usually begins with a physical examination. Based on the findings, healthcare professionals may recommend further imaging and laboratory tests to confirm the diagnosis and determine the extent of the disease.

Imaging tests

Imaging tests create detailed pictures of the body to show the location, size, and spread of the tumor. Common imaging tests for Ewing sarcoma include:

• X-ray

• Magnetic resonance imaging (MRI)

• Computerized tomography (CT) scan

• Bone scan

• Positron emission tomography (PET) scan

These tests help identify bone and soft tissue involvement and whether the cancer has spread to other parts of the body.

Removing a sample of cells for testing

A biopsy is performed to remove a small sample of tissue for laboratory testing. This can be done in two ways:

• Needle biopsy: A needle is inserted through the skin and into the tumor to collect tissue.

• Surgical biopsy: A minor surgery is performed to remove a small part of the tumor.

The biopsy sample is examined under a microscope to confirm whether it is Ewing sarcoma. Additional tests are performed on the tissue to gather more details about the cancer cells.

A biopsy is essential for confirming the diagnosis and helping your healthcare team design an appropriate treatment plan.

Testing the cancer cells for DNA changes

Cancer cells from the biopsy are tested in the lab to check for specific DNA changes. Most Ewing sarcoma tumors involve changes in the EWSR1 gene, which often fuses with another gene called FLI1, forming EWS-FLI1.

Detecting this genetic change helps confirm the diagnosis and provides valuable information about the biology of the tumor.

Treatment

Ewing sarcoma treatment usually involves a combination of chemotherapy, surgery, and sometimes radiation therapy or targeted therapy. The treatment plan depends on the location, size, and spread of the tumor.

Chemotherapy

Chemotherapy uses strong medicines to kill cancer cells. It is often the first step in treatment to shrink the tumor and make it easier to remove surgically or target with radiation therapy.

After surgery or radiation, chemotherapy may be continued to destroy any remaining cancer cells. In advanced cases, chemotherapy can also help relieve pain and slow cancer growth.

Surgery

Surgery aims to remove all cancerous tissue. Depending on the tumor’s location and size, surgery might involve removing a small section of bone or soft tissue. In rare cases, amputation of a limb may be necessary.

Whenever possible, surgeons use limb-sparing techniques to preserve function and reduce disability. The surgical approach depends on tumor size, location, and how well the cancer responds to chemotherapy.

Radiation therapy

Radiation therapy uses powerful energy beams—such as X-rays or protons—to destroy cancer cells. It may be used after surgery to eliminate remaining cancer cells or as an alternative to surgery when an operation could damage nearby organs.

For advanced Ewing sarcoma, radiation therapy may also help slow cancer growth and relieve pain.

Targeted therapy

Targeted therapy uses medicines designed to attack specific weaknesses in cancer cells. By blocking key molecules involved in tumor growth, these treatments can stop cancer cells from multiplying. Researchers are studying targeted therapies for Ewing sarcoma, especially for cases where the cancer returns or doesn’t respond to standard treatments.

Clinical trials

Clinical trials test new and innovative treatments for Ewing sarcoma. They provide patients access to emerging therapies that might not yet be widely available. Discuss with your healthcare team whether joining a clinical trial could be a suitable option.