Medically Reviewed by Jennifer Robinson, MD on March 18, 2021
Ewing’s sarcoma is a very rare type of cancerous tumor that grows in your bones or the soft tissue around your bones, such as cartilage or the nerves. It usually affects people from the ages of 10 to 20 and has a high rate of being cured.
Ewing’s sarcoma affects about 200 children and young adults every year in the United States and shows up slightly more often in males. While adults can get Ewing’s sarcoma, it is uncommon. It mostly affects white people and has rarely been found in African Americans or Asian Americans.
Types of Ewing’s Sarcoma
The kind of Ewing’s sarcoma you might have can be seen where the tumor forms. The pelvis is where it most often starts, followed by the femur (or thigh bone). Regardless of where it starts, it can spread to other bones, bone marrow, and even vital organs such as the lungs, heart, and kidneys. Some types are:
Bone tumor: 87% of Ewing’s sarcoma happens in the bone, often appearing in the thigh bones, pelvis, ribs, or shoulder blades. But the tumor can appear in any bone.
Soft tissue (extra-osseous) tumor: This type of tumor affects the soft tissues around your bones, such as cartilage or muscle. It’s rarely found in the soft tissue of the arms, legs, head, neck, chest, and abdomen.
Peripheral primitive neuroectodermal tumor (pPNET): This type of tumor is found in the nerves and can be detected in many parts of the body.
Askin tumor: This is a type of pPNET tumor that’s found in the chest.
Causes
It’s unclear what causes Ewing’s sarcoma, as it doesn’t appear to run in families. So far, research suggests that it’s not related to exposure to radiation, chemicals, or other outside things in the environment.
It appears that the cell DNA changes after birth, leading to Ewing’s sarcoma. Why that happens remains unknown.
One possibility is that it could be a second cancer in people who’ve been treated with radiation for another type of cancer.
Symptoms
When you have Ewing’s, you may feel pain, swelling, or stiffness in the area of the tumor (arms, legs, chest, back, or pelvis) for weeks or months. This could get mistaken for bumps and bruises. In children, you might mistake it for sports injuries.
Other symptoms include:
● A lump near skin that feels warm and soft to the touch
● Constant low fever
● Limping because your legs hurt
● Bone pain that gets worse when you exercise or during the night
● Broken bones without an obvious cause
● Weight loss
● Always being tired
● Paralysis or loss of bladder control if the tumor is near your spine
When you see symptoms, you should contact your doctor so they can do a diagnosis. As with many types of cancer, the earlier you have it detected and treated, the better.
Diagnosis and Tests
Your doctor has a number of tests to see whether you have Ewing’s sarcoma. If you do, they will also try to find out whether the tumor has spread and to what extent. This is known as staging.
Physical exam: Your doctor will start out with this, especially checking around the painful areas for signs such as lumps, redness, and swelling.
X-rays: These take an image of a certain area of your body where a tumor is suspected. If the X-rays show any problems, your doctor may order other imaging tests.
Bone scan: This is used to find out whether cancer cells are in your bones. A small amount of radioactive dye is injected into one of your veins. The dye will collect in the bones that have tumors. When you lie beneath a bone scanner, the doctor will be able to detect where the radioactive dye has gathered.
Magnetic resonance imaging (MRI): For this test, you lie flat and still on a surface that slides you inside the tube-shaped MRI machine. It uses magnetic field and radio waves to create a detailed, 3-D image inside your body.
Computed tomography scan(CAT/CT scan): Like you would do for an MRI, you lie flat inside a CT scanner, which takes X-ray pictures while linked to a computer. The MRI is generally preferred over the CT scan.
Positron emission tomography scan (PET scan): You lie inside the PET scanner while a technician injects a small amount of radioactive sugar into a vein. The cancer cells show up brighter in the scan because they use more sugar than healthy cells.
Blood test: This cannot confirm Ewing’s sarcoma. But a complete blood count, or CBC, checks how many red blood cells, white blood cells, and how much hemoglobin (a protein that carries oxygen) you have.
Biopsy: Your doctor removes a piece of your tissue with either a needle or during surgery. That sample is viewed under a microscope to see if it’s cancerous. This can confirm whether you have Ewing’s sarcoma.
Bone marrow aspiration and biopsy: Your doctor numbs an area of your body, usually in the hips. They insert a needle and takes a small piece of bone marrow and bone to exam under a microscope.
Treatments
What kind of treatment you get will depend on several things. They include:
- The size of your tumor
- Where it has spread
- Your overall health
- Your preferences, which you should talk over with your doctor
Treatment options include:
Chemotherapy: This is usually the first step. With this option, medicines are used to kill cancer cells and stop them from growing. They can be injected into your bloodstream. Your doctor may use more than one type of chemotherapy at a time or combine this with surgery and radiation.
Surgery: Your doctor will try to remove the tumor to stop its spread. In some cases, they may have to amputate an arm or leg if the tumor has spread a lot.
Radiation: In this therapy, a technician will use X-rays and other types of radiation to kill the cancer cells. This can be done using machines outside the body to deliver the dose, or through needles and tubes sent right to the tumor.
If the treatment works, you will still need to follow up with your doctor for many years. Ewing’s sarcoma can return even a decade after the diagnosis.
Children with Ewing’s sarcoma that hasn’t spread have cure rates as high as 80%. It’s much lower in cases where the tumor has spread. It’s important to see a doctor right away if you see symptoms in your child.
