Ewing Sarcoma (Most often found in pediatric patients)

Ewing sarcoma is cancer that occurs primarily in the bone or soft tissue, most often in children between the ages of 10 and 20. It usually develops during puberty when bones are growing rapidly. Although Ewing sarcoma can develop in any bone, it usually affects the thigh, shin and upper arm bones. The pelvis bones are also often affected. Ewing sarcoma cells can also metastasize (spread) to other areas of the body, including the bone marrow, lungs, kidneys, heart, adrenal glands and other soft tissues.

Types & Stages 

The main types of Ewing tumors are:

• Ewing sarcoma of bone. Ewing sarcoma that starts in a bone is the most common tumor in this family. This type of tumor was first described by Dr. James Ewing in 1921, who found it was different from the more common bone tumor, osteosarcoma.  
   
• Extraosseous Ewing tumor (EOE). Extraosseous Ewing tumors start in soft tissues around bones, but they look and act very much like Ewing sarcomas in bones. They are also known as extraskeletal Ewing sarcomas.
   
• Peripheral primitive neuroectodermal tumor (PPNET). This rare childhood cancer also starts in bone or soft tissue and shares many features with Ewing sarcoma of bone and EOE. Peripheral PNETs that start in the chest wall are known as Askin tumors.

Signs and Symptoms 

When Ewing sarcoma signs and symptoms happen in and around a bone, they might include:

• A lump in the arm, leg, chest or pelvis
• Bone pain
• Break in a bone, also called a fracture
• Pain, swelling or tenderness near the affected area
• Fever
• Losing weight without trying
• Tiredness

Diagnosis 

Tests and procedures to diagnose Ewing sarcoma include:  

• Imaging tests. These may include:
   
  • X-rays. A diagnostic test that uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film.
     
  • Computerized tomography scan (CT or CAT scan). A diagnostic imaging procedure that uses a combination of X-rays and computer technology to produce cross-sectional images (often called slices), both horizontally and vertically, of the body.  
     
  • Magnetic resonance imaging (MRI). A diagnostic procedure that uses a combination of large magnets, radiofrequencies, and a computer to produce detailed images of organs and structures within the body.
     
  • Bone scans. A whole-body bone scan is a nuclear medicine test to check your bones for issues or changes. You receive an injection of a substance called a radiotracer. The radiotracer collects in areas of irregular activity and highlights these areas on an imaging scan.  
     
  • Positron emission tomography scan (PET scan). An imaging test that uses a radioactive drug called a tracer — most often injected into a vein in your hand or arm — to show both typical and atypical metabolic activity. The PET images are typically combined with CT or MRI and are called PET-CT or PET-MRI scans.  
     
• Biopsy. A biopsy is needed to confirm a Ewing sarcoma diagnosis. A procedure to remove some cells for testing. The sample might be removed with a needle put through the skin. Or the sample might be taken during surgery to remove the cancer. The type of biopsy depends on the cancer's location.

Treatment 

Ewing sarcoma treatment options include:

• Chemotherapy. Chemotherapy is sometimes used as the first treatment for Ewing sarcoma. The medicines may shrink the cancer. That makes it easier to remove the cancer with surgery or target with radiation therapy. After surgery or radiation therapy, chemotherapy treatments might be used to kill any cancer cells that might remain. For advanced cancer that spreads to other areas of the body, chemotherapy might help relieve pain and slow the growth of the cancer.
   
• Surgery. The goal of surgery is to remove all the cancer cells. Surgery for Ewing sarcoma might mean removing a small portion of bone and some surrounding tissue. Rarely, it might mean removing the affected arm or leg. Surgery on an arm or leg might affect the way you can use that limb. Surgeons carefully plan the surgery to minimize this risk, when possible. Whether surgeons can remove all the cancer without removing the arm or leg depends on several factors. These include the size of the cancer, where it is and whether chemotherapy helps shrink it.
   
• Radiation therapy. Radiation therapy might be suggested after surgery to kill cancer cells that remain. Radiation therapy might be used instead of surgery if an operation is not possible or if it is likely to hurt nearby organs. For example, if the surgery might cause loss of bowel or bladder control, radiation might be used instead. For advanced Ewing sarcoma, radiation therapy can slow the growth of the cancer and help relieve pain.
   
• Targeted therapy. A treatment that uses medicines that attack specific ways that cancer cells can grow. By blocking these specific things in the cells, targeted treatments can cause cancer cells to die. For Ewing sarcoma, researchers are looking at using targeted therapy when the cancer comes back or does not respond to other treatments. 

Causes & Risk Factors 

Risk factors for Ewing sarcoma include:

• Age. Ewing sarcoma can happen at any age, but it is more likely to happen in children and young adults.
   
• European ancestry. Ewing sarcoma is more common in people of European ancestry. It's much less common in people of African and East Asian ancestry.

Screening 

There is no routine screening for Ewing sarcoma.