Ewing Sarcoma vs. Other Bone Cancers: Key Differences to Know
Ewing sarcoma is a type of cancer that starts in your bones or nearby soft tissues. It most commonly develops in your pelvis, axial skeleton (central bones such as your ribs and spine) and long bones, especially your femur, or thigh bone.
Children and young adults are most likely to develop Ewing sarcoma, with the highest number of cases occurring between the ages of 10 and 15. Around one-third of cases occur in children under 10, while another third appear in young adults older than 20. Ewing sarcoma is more common in boys than in girls.
Learning that you or your child has Ewing sarcoma can be frightening. Fortunately, while this disease grows quickly, researchers have identified effective treatment strategies. Let’s learn more about Ewing sarcoma, how it differs from other bone and soft tissue sarcomas, and why advanced care makes a difference for this rare disease.
What Causes Ewing Sarcoma?
Researchers do not understand why some people develop Ewing sarcoma. The disease is relatively uncommon, with only about 200–300 cases occurring in the U.S. each year. Ewing sarcoma cells tend to have rearrangements of specific genes, which may lead to mistakes in transcription, or copying genetic information. However, the source of these rearrangements remains unknown.
Researchers have not located any environmental or genetic risk factors for Ewing sarcoma. The genetic changes leading to Ewing sarcoma happen after birth, rather than being inherited from your parents.
Ewing Sarcoma Progression
Ewing sarcoma cancer cells generally start in your bones, though they can also begin in soft tissue anywhere in your body. From there, the disease may spread to your lungs, other bones or bone marrow.
People with metastatic Ewing sarcoma (disease that has spread to other parts of the body) tend to have worse outcomes. However, about 25% of the time, Ewing sarcoma returns even when doctors diagnose it before it can spread.
What Makes Ewing Sarcoma Unique Among Childhood Cancers?
Ewing sarcoma is the second most common type of childhood bone cancers. It has several unique factors that distinguish it from similar diseases.
Its diversity, for one thing, sets it apart. Ewing sarcoma has many types, including:
- Ewing sarcoma of the bone, the most common kind of Ewing sarcoma. Other Ewing sarcomas are grouped together because their cells resemble those of Ewing sarcoma bone disease.
- Extraosseous Ewing tumors, which start in soft tissues near bones
- Peripheral primitive neuroectodermal tumors, which can start in bone or soft tissue. These include Askin’s tumor, which grows in the bones in your chest.
Ewing Sarcoma vs. Osteosarcoma
Osteosarcoma, the most common type of childhood bone cancer, always occurs in bones. Osteosarcoma features tumors containing immature bone cells, and it is more difficult to treat successfully than Ewing sarcoma.
Ewing Sarcoma vs. Other Soft-Tissue Sarcomas
Other soft tissue sarcomas are found in soft tissues throughout the body, including blood vessels, fat and nerves. Unlike with Ewing sarcoma, there are known risk factors for soft tissue sarcomas, including previous radiation treatment and family cancer syndromes.
Early Symptoms of Ewing Sarcoma
One thing Ewing sarcoma does share with other osteosarcomas and soft tissue sarcomas is early symptoms. Parents often bring their child to the doctor when they complain of pain and a lump.
Symptoms of Ewing sarcoma include:
- Bone pain when you don’t remember you or your child having an injury
- Bumps or lumps not associated with an injury, especially ones that feel soft or warm
- Flu-like symptoms, including body aches, fatigue and fever
- Pain, swelling and tenderness near a bump
Ewing sarcoma may not initially cause painful symptoms. Always mention any unexplained bumps to your doctor or your child’s pediatrician, even when there isn’t pain or swelling. Also be alert to fractures associated with a slight injury, as tumors can weaken bones and make them easier to break.
How Do Doctors Diagnose Ewing Sarcoma?
After a physical exam, doctors may use several different approaches to see whether you or your child has Ewing sarcoma and to find out whether the disease has spread.
- Imaging tests: Imaging studies, including X-ray, MRI and CT scans with contrast, can reveal tumors in the bones and soft tissue.
- Biopsy: Depending on where doctors suspect the cancer has spread, biopsy sites may include bones, soft tissue and bone marrow. An orthopedic surgeon may perform an open bone biopsy.
Ewing Sarcoma Treatments
Advanced treatments have extended life for children and young adults with Ewing sarcoma, especially in its early stages. Surgery to remove the tumor, along with chemotherapy and radiation, is typically performed to treat Ewing sarcoma.
Today, orthopedic surgeons can often remove bone tumors without amputation. Other surgeries may be used to decompress vertebrae that are pressing into your spinal cord, remove partial or entire bones of the chest area or, rarely, to amputate an entire limb.
Other therapies include:
- Chemotherapy: You receive medication that kills cancer cells. One form of chemotherapy used for Ewing sarcoma is isolated limb perfusion. In this treatment, doctors temporarily separate the circulation of one limb from the rest of your body so they can deliver especially strong chemotherapy.
- Immunotherapy: Immune checkpoint inhibitors can help your immune system recognize and attack cancer cells.
- Radiation: Radiotherapy may be used to control cancer that can’t be operated on. You may also receive radiation therapy before surgery to shrink a tumor or after surgery to help destroy any remaining cancer cells.
- Targeted therapies: Medications that affect specific aspects of cancer cells may be helpful if your disease can’t be removed surgically and hasn’t responded to other treatments.
Fertility Counseling
Because Ewing sarcoma treatment side effects can damage the reproductive system, you may receive fertility counseling for yourself or your child. Patients may have the option to preserve sperm or eggs so they can have children after cancer treatment.
Prostheses and Growth Surgeries
While they don’t treat cancer, high-quality orthopedic tools and treatments make a huge difference in the lives of growing children. Orthopedic surgeons offer advanced procedures and prostheses to replace joints, help surgically shortened limbs grow along with your child, and otherwise keep your young person on the move.
A Unique Cancer Needs Specialized Care
If you or your child is facing a diagnosis of Ewing sarcoma, you need an experienced care team on your side.
Capital Health Cancer Center offers:
- Advanced orthopedic oncology procedures, including prophylactic repair to strengthen bones weakened by cancer
- Clinical trial access through the National Cancer Institute
- Multidisciplinary care in partnership with the Rothman Orthopaedic Institute
Don’t struggle with a diagnosis of Ewing sarcoma alone—bring your questions to compassionate experts who can offer leading-edge treatments while providing long-term support for you and your family.
Schedule a consultation with an oncologist at Capital Health Cancer Center.