Ewing's Sarcoma

Ewing's sarcoma is a rare type of tumor *

Tom's Story: “It Felt like a Pulled Muscle”

Tom woke up one morning with pain like a pulled muscle in his right leg, about halfway up the upper leg bone. He noticed a tiny lump there, but he figured he got a bump when he was playing basketball the day before, and he thought it would go away. When the lump and the pain were still there the next day, he mentioned it to his mother. She told him to wait a few days and see if it got better. It did not. She scheduled an appointment with his doctor, who examined Tom and ordered several tests, including an x-ray. Not long afterward, the doctor called to tell her that Tom had Ewing's sarcoma and should begin treatment right away. Tom was lucky because the doctor found the tumor when it was still very small. Surgery was scheduled and the tumor was removed. Tom underwent physical therapy and had to stop playing basketball and other sports, but he recovered and returned to school.

What Is Ewing's Sarcoma?

Ewing's sarcoma is a rare type of cancer in which a malignant * tumor forms in bone or soft tissues near bone. Usually these tumors appear in the middle of the upper arm or upper leg bone, although they may show up elsewhere in the body, such as the ribs or the hip bones. People with this disease usually experience pain, tenderness, or swelling at the site of the tumor. Sometimes, however, individuals have a tumor for many months before they have any pain or swelling. Ewing's sarcoma is a dangerous cancer because it can spread to other areas of the body. A number of treatments are available and can be quite successful, especially if doctors find the tumors and diagnose Ewing's sarcoma early, before it has spread. Some medical professionals once used the name primitive neuroectodermal (ner-o-ek-toe-DERM-ul) tumor (PNET), sometimes called peripheral neuroepithelioma, when referring to Ewing's sarcoma. However, although PNET is similar to Ewing's sarcoma, it is not the same. As of 2015, medical professionals referred to PNET as being in the Ewing's family of tumors or a tumor of the Ewing's family (TEF). According to the American Cancer Society, Ewing's sarcoma is by far the most common tumor in this family and makes up 87 percent of TEFs.

Forearm bones of a patient diagnosed with Ewing's sarcoma, the second most common bone tumor among children and teens. Half of all cases involve flat or long bones, such as in the forearm or leg.

Forearm bones of a patient diagnosed with Ewing's sarcoma, the second most common bone tumor among children and teens. Half of all cases involve flat or long bones, such as in the forearm or leg.
Mike Devlin/Science Source.

Ewing's sarcoma results from an unusual arrangement, called trans-location, of two chromosomes * . Chromosomes are packages of DNA * , as well as various proteins, that are found in a cell's nucleus. They carry an individual's genes *

How Common Is Ewing's Sarcoma?

Many types of cancer affect adults more frequently than children, but this is not the case with Ewing's sarcoma. This tumor occurs more often in teenagers than in people of other age groups. Nonetheless, the American Cancer Society reports that Ewing's sarcoma is rare. According to the American Cancer Society, about 225 children and adolescents in the United States each year find out they have a TEF. Of those who are diagnosed with a TEF, four-fifths are white, nearly two-thirds are between the ages of 10 and 20 years old, and more than 50 percent are male. All told, Ewing's sarcoma makes up 2 percent of all teen cancers. It is not contagious.

How Do People Know They Have Ewing's Sarcoma?

A typical symptom of Ewing's sarcoma is pain, sometimes accompanied by swelling, at the site of the tumor. Depending on the position of the tumor and how near it is located to nerves, the pain can be quite severe. Often, the tumor is located in the middle of the upper arm bone (the humerus) or the upper leg bone (the femur), but tumors may also be located in the area between the two hips (the pelvis), in the chest, or in the back. A lump may appear at the site of the tumor, and the patient may feel warmth at the site. Also, tumors that are located near nerves can cause patients to feel numbness or tingling and can occasionally cause paralysis * or incontinence * .

In addition to these symptoms, a patient may experience a fever, fatigue, little or no appetite, and weight loss. Occasionally individuals with Ewing's sarcoma break a bone, although they have had no accidents or other injuries that would cause a broken bone. Such a break happens because the disease weakens bone. Sometimes individuals have no other symptoms of a tumor until a bone breaks, and this broken bone is what leads a doctor to diagnose the tumor. In some cases, individuals may have a tumor for many months before it begins to cause symptoms because a tumor starts out small and grows over time. Individuals may have no symptoms when the tumor is small but may begin to experience pain or swelling when the tumor gets larger.

How Do Doctors Diagnose and Treat Ewing's Sarcoma?


After hearing about the symptoms an individual is having, a doctor may order a number of tests to look more closely at the affected area, including x-rays, bone scans, magnetic resonance images * , and CT scans * . If the results point to a tumor, the doctor then conducts a biopsy (BY-op-see) to remove a tiny piece of tissue. A medical professional then looks at the sample carefully using a microscope to determine whether there is evidence of cancer. In addition, doctors may conduct tests to look at chromosomes 11 and 22 to discover whether they are translocated.

After Ewing's sarcoma is diagnosed, the doctor may order additional tests to ascertain whether cancer has spread from the site of the tumor to other areas of the body. This information helps the doctor decide how to treat the disease. If Ewing's sarcoma has spread, it usually moves into the lungs first. It may also move into other bones or into the bone marrow * .


The treatment for Ewing's sarcoma usually involves chemotherapy * , radiation therapy * , and surgery. Radiation or surgery is the typical treatment for tumors that have not spread elsewhere. Usually a doctor's first choice is to remove the tumor surgically. Sometimes, however, doing so proves difficult because the tumor is located in an area where surgical removal may harm the patient's use of an associated body part. In this case, doctors may opt for radiation therapy to kill the tumor cells, or a combination of surgery and radiation. For instance, if surgery does not remove the tumor entirely, then radiation may be prescribed to treat the leftover tumor. Also, doctors sometimes use radiation to make sure the cancer does not return.

Doctors turn to chemotherapy especially when the tumor has spread, because chemotherapy kills both tumor cells, additional cancer cells, and other cells throughout the body. Doctors monitor the tumor continuously during the patient's course of chemotherapy to determine whether the tumor is shrinking. When the tumor has shrunk sufficiently, a surgeon may remove it.

During surgery for Ewing's sarcoma, doctors remove the tumor and make repairs at the tumor site. These repairs may include reconstructing the bone or joint, and perhaps using artificial joints or bone grafts * . A bone graft is new bone or another material, such as a metal rod, that replaces the bone that was removed during surgery. These artificial joints and bone grafts, along with physical therapy after the surgery, help individuals return to many of their normal activities. Doctors tell patients to avoid athletic exercise and any other movements that may put stress on the repaired bones or joints. Occasionally patients need additional operations later in life to keep bones and joints functioning properly. Subsequent operations occur often among children, whose bones are still developing. Doctors may need to do several surgeries to lengthen the repaired bone as the child grows.

Until the late 20th century, amputation * of an arm or leg was a common treatment for Ewing's sarcoma. With the improvement in chemotherapy treatments and surgery, doctors can remove the tumors in most patients without having to amputate a limb. However, some patients with Ewing's sarcoma must have a limb amputated. After an amputation, individuals typically receive an artificial limb, called a prosthesis, and work with physical and occupational therapists to learn how to use it to its greatest advantage.

Even after individuals have had the tumor removed, they still have to return for follow-up care to ensure that the tumor is indeed gone. This care may include additional diagnostic tests, such as x-rays or bone scans. Overall, the survival rate for Ewing's sarcoma varies greatly and depends, to a great extent, on many factors, including the following:

  • The size of the tumor
  • The location of the tumor
  • Whether the cancer spread to other parts of the body
  • How well the tumor responds to chemotherapy or other treatment
  • The overall health of the patient

In addition to these treatments, a doctor may recommend psychological therapy to help individuals cope with the disease and its effect on their life. Teenagers especially may benefit from psychological therapy because they are dealing with adolescent issues that are also quite challenging.

Can Ewing's Sarcoma Be Prevented?

As of 2015, researchers knew of no way to prevent people from developing Ewing's sarcoma.

See also Cancer: Overview • Tumor


Books and Articles

National Cancer Institute. 21st Century Pediatric Cancer Sourcebook: Ewing Sarcoma Family of Tumors (Bone, PNET, Extraosseous): Clinical Data and Practical Information for Patients, Families, and Physicians. Bethesda, MD: Progressive Management, 2011.


American Cancer Society. “Ewing Family of Tumors.” http://www.cancer.org/cancer/ewingfamilyoftumors/detailedguide/index (accessed April 14, 2016).

The Liddy Shriver Sarcoma Initiative. “Ewing's Sarcoma Family of Tumors (ESFT).” http://sarcomahelp.org/ewings-sarcoma.html (accessed March 4, 2016).


American Academy of Orthopaedic Surgeons. 6300 North River Rd., Rosemont, IL, 60018-4262. Telephone: 847-823-7186. Website: http://www.aaos.org/home.asp (accessed March 4, 2016).

American Cancer Society. 250 Williams St. NW, Atlanta GA 303031034. Toll-free: 800-ACS-2345. Website: http://www.cancer.org (accessed March 4, 2016).

National Cancer Institute. Public Inquiries Office, 6116 Executive Blvd., Room 3036A, Bethesda, MD, 20892-8322. Toll-free: 800-4-CANCER. Website: http://www.cancer.gov (accessed March 4, 2016).

* tumor (TOO-mor) is an abnormal growth of body tissue that has no known cause or physiologicpurpose. A tumor may or may not be cancerous.

* malignant (ma-LIG-nant) is a condition that is severe and progressively worsening.

* DNA, or deoxyribonucleic acid (dee-OX-see-ry-bo-nyoo-klay-ik AH-sid), is the specialized chemical substance that contains the genetic code necessary to build and maintain the structures and functions of living organisms.

* genes (JEENS) are chemical structures composed of deoxyribonucleic acid (DNA) that help determine a person's body structure and physical characteristics such as hair or eye color. Inherited from a person's parents, genes are contained in the chromosomes found in the body's cells.

* chromosomes (KRO-mo-somz) are threadlike chemical structures inside cells on which the genes are located. There are 46 chromosomes (23 pairs) in normal human cells. Genes on the X and Y chromosomes (known as the sex chromosomes) help determine whether a person is male or female. Females have two X chromosomes; males have one X and one Y chromosome.

* paralysis (pah-RAH-luh-sis) is the loss or impairment of the ability to move some part of the body.

* incontinence (in-KON-ti-nens) is loss of control of urination or bowel movement.

* magnetic resonance imaging (or MRI) uses magnetic waves, instead of x-rays, to scan the body and produce detailed pictures of the body's structures.

* CT scans, computed tomography (to-MOG-ra-fee) scans, or CAT (computerized axial tomography) scans, use computers to view structures inside the body.

* bone marrow is the soft tissue inside bones where blood cells are made.

* chemotherapy (KEE-mo-THER-apee) is the treatment of cancer with powerful drugs that kill cancer cells. * radiation therapy is a treatment that uses high-energy radiation from x-rays and other sources to kill cancer cells and shrink cancerous growths.

* grafts are tissue or organ transplants.

* amputation (am-pyoo-TAY-shun) is the removal of a limb, other appendage, or outgrowth of the body.

Disclaimer:   This information is not a tool for self-diagnosis or a substitute for professional care.

(MLA 8th Edition)