What Is Ewing's Sarcoma?

Medically Reviewed by Poonam Sachdev on November 17, 2023
5 min read

Ewing’s sarcoma is a rare type of cancerous tumor that grows in the bones or the soft tissue around the bones, such as cartilage. It is most common in children and young adults, but anyone can have it. 

Ewing’s sarcoma affects about 200 children and young adults every year in the United States. It's slightly more common in males than females. It mostly affects people of European descent and has rarely been found in people of African or Asian descent.

Ewing's sarcoma vs. osteosarcoma

Osteosarcoma is another type of cancerous bone tumor that mainly affects young people. It is more common than Ewing's sarcoma. Osteosarcoma usually grows in bones around the knee, and Ewing's sarcoma usually grows in the bones of the thighs, pelvis, ribs, and upper arms. These two types of bone cancer are treated in a similar way.

 

 

There are four types of Ewing's sarcomas based on the place where the tumor forms:

Bone tumor: 87% of Ewing’s sarcomas are in the bone, usually the thigh bones, pelvis, ribs, or upper arms. But the tumor can grow in any bone.

Soft tissue tumor: This type of tumor grows in the soft tissues around your child's bones, such as cartilage.

Peripheral primitive neuroectodermal tumor (PNET): This type of tumor is found in the nerves and can grow in many areas of your child's body.

Askin tumor: This is a type of PNET tumor that’s found in your child's chest wall.

All types of Ewing's sarcoma can spread to other areas of your child's body, such as their bones, bone marrow, and lungs.

Doctors don't know what causes Ewing's sarcoma. It doesn't run in families, and it’s not caused by anything related to diet, lifestyle, or environment.

Research shows that changes in a cell's DNA that happen after your child's birth most likely lead to Ewing’s sarcoma. Scientists still don't understand why that happens.

 

With Ewing's sarcoma, your child may feel pain, swelling, or stiffness in the area of the tumor. This is sometimes mistaken for bumps and bruises from playing sports.

Ewing's sarcoma might also cause your child to:

  • Have a lump near the skin that feels warm and soft to the touch
  • Have a constant low fever
  • Limp because their legs hurt
  • Have bone pain that gets worse when they exercise or during the night
  • Have broken bones without an obvious cause
  • Lose weight without trying
  • Feel tired for no reason

Your doctor has a number of tests to see whether your child has Ewing’s sarcoma. If they do, your doctor will also try to find out whether the tumor has spread. 

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 the area of your child's body where the tumor might be. 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 child's bones. A small amount of radioactive dye is injected into your child's veins. The dye will collect in the bones that have tumors. When your child lies under a bone scanner, the doctor will be able to detect where the radioactive dye has gathered.

Magnetic resonance imaging (MRI): For this test, your child lies flat and still on a surface inside a tube-shaped machine. It uses a magnetic field and radio waves to create a detailed 3D image of the inside of your child's body.

Computed tomography scan (CT scan): Similar to the MRI, your child lies flat inside a CT scanner, which takes X-ray pictures while linked to a computer. CT scans are more detailed than X-rays but not as detailed as MRIs.

Positron emission tomography scan (PET scan): A technician injects a small amount of a radioactive sugar into your child's vein. Your child then lies inside the tube-like PET scanner machine. The cancer cells show up brighter in the scan because they use more sugar than healthy cells.

Biopsy: Your doctor removes a small piece of tissue from your child's tumor. The sample is viewed under a microscope to see if it’s cancerous. 

Bone marrow aspiration and biopsy: Your doctor takes a small piece of bone marrow from your child's bone to exam under a microscope. This is usually used to see if the cancer has spread.

What kind of treatment your child has depends on:

  • The size of their tumor
  • Where it has spread
  • Their overall health
  • Your preferences, which you should talk over with your doctor

Treatment options include:

Chemotherapy: This is usually the first step. During chemotherapy, you're child takes medication either by pill or shot to shrink the tumor. Your child's doctor may use more than one type of chemotherapy at a time or combine this with surgery and radiation.

Surgery: Surgery usually follows chemotherapy. Your doctor will try to remove the tumor to stop its spread. In some cases, they may have to remove 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. Ewing's sarcoma radiology can be done using machines outside the body to deliver the dose, or through needles and tubes sent right to the tumor.

 

Survival rates for Ewing's sarcoma depend on if your child's cancer has spread. According to the American Cancer Society, if the cancer hasn't spread, your child has an 82% chance of living at least another 5 years. If the cancer has spread, the survival rate is between 39% and 71%, depending on how far it has spread.

It's important to note that these are only estimates. As treatments improve, so do the survival rates. Ewing's sarcoma treatments have become much more effective in recent years. 

Your child will need follow-up care with your doctor for many years after treatment. There is a chance of recurrence, and the treatments for the cancer can have effects that show up later in your child's life.