What Is the Survival Rate of Ewing Sarcoma?

The five year survival rate for Ewing Sarcoma, a rare cancer of the arm bones, ranges from above 80% for localized tumors treated early and under 40% for people in whom the sarcoma has metastasized.

Ewing sarcomas are rare types of cancerous tumors that originate in the long bones of the arms and legs, pelvis, or chest or in nearby soft tissues. Ewing tumors can develop at any age, but they mainly affect children and teens. 

There are three main types of Ewing tumors:

• Ewing sarcoma of bone
  • The most common type of Ewing tumor 
• Extraosseous Ewing tumor (EOE)
  • Starts in soft tissues around bones, but they look and act very much like Ewing sarcomas in bones
  • Also called extraskeletal Ewing sarcomas
• Peripheral primitive neuroectodermal tumor (PPNET)
  • Rare childhood cancer 
  • Also starts in bone or soft tissue and has many features in common with Ewing sarcoma of bone and EOE
  • PPNETs that start in the chest wall are known as Askin tumors

Symptoms of Ewing sarcoma may include:

• Pain in the area of the tumor
  • Ewing tumors occur most often in the pelvis (hip bones), the chest wall (ribs or shoulder blades), and legs (primarily in the middle of the long bones)
• Swelling, frequently in the arms or legs
• A lump under the skin that is often soft and feels warm
• Bone fracture that occurs without a known cause
• Tumors near the spine may cause 
  • Back pain 
• Weakness, numbness, or paralysis in the arms or legs

If the tumor has spread to other parts of the body (metastasized), symptoms may include: 

• Fever
• Night sweats
• Tiredness
• Weight loss
• Shortness of breath (from tumors that have spread to the lungs)

The cause of Ewing sarcoma is a genetic change, most often an abnormal rearrangement of genetic material (reciprocal translocation) that causes two genes, the EWSR1 gene on chromosome 22 and the FLI1 gene on chromosome 11, to fuse together, which leads to abnormal cell growth. 

The genetic change is not inherited and the cause of the reciprocal translocation is unknown. 

Risk factors for developing Ewing sarcoma include: 

• Race/ethnicity
• Much more common among whites (either non-Hispanic or Hispanic) than among Asian Americans or African Americans 
• Gender
• Slightly more common in males than in females
• Age
• Most common in teens 

Ewing sarcoma is diagnosed with a physical examination and the following tests: 

• Imaging studies
  • X-rays
  • Magnetic resonance imaging (MRI) scan
  • Computed tomography (CT) scan
  • Bone scan
  • Positron emission tomography (PET) scan
• Biopsy 
  • Tumor biopsy
  • Bone marrow aspiration and biopsy
• Blood tests
  • Complete blood count (CBC)
  • Blood chemistry 
  • Lactate dehydrogenase (LDH) enzyme

Treatment for Ewing sarcoma depends on the size and location of the tumor, and whether or not it has spread to other parts of the body (metastasized).  

Treatments for Ewing sarcoma may include one or more of the following: 

• Chemotherapy
  • VDC/IE (or VAC/IE), a regimen that alternates between 2 combinations of drugs given every 2 to 3 weeks:
    • The first set of drugs includes vincristine (Oncovin), doxorubicin (Adriamycin), and cyclophosphamide
    • Once the patient recovers, a combination of ifosfamide (Ifex) and etoposide (Vepesid) is given
  • Pazopanib (Votrient) is approved by the Food and Drug Administration (FDA) as an orphan product for treatment of Ewing sarcoma (orphan products are used for very rare conditions)
• High-dose chemotherapy and stem cell transplant 
• Surgery
  • Tumors in the arms or legs
    • Removal of part or all of the affected bone while leaving the arm or leg basically intact (limb-sparing surgery)
    • Removal of part or all of the limb (amputation) 
  • Tumors in the chest wall or pelvis (hip bones)
    • Removal of the diseased area and removal of nearby ribs
• Radiation therapy
  • Three-dimensional conformal radiation therapy (3D-CRT)
  • Intensity modulated radiation therapy (IMRT)
  • Conformal proton beam radiation therapy

Life expectancy for Ewing sarcoma is often expressed in 5-year survival rates, that is, how many people will be alive 5 years after diagnosis. 

Ewing sarcoma 5-year survival rates:

• Localized (no sign that the cancer has spread outside of the bone or other area where it started): 82%
• Regional (cancer has spread outside the bone or other area and into nearby structures, or it has reached nearby lymph nodes): 67%
• Distant (cancer has spread to distant parts of the body, such as to the lungs or to bones in other parts of the body): 39%