What is Ewing’s sarcoma?

Ewing’s sarcoma is a malignant tumor of the bone. It may occur in any bone, however it most commonly affects the pelvis, tibia (shin), fibula (shin), and femur (thigh). Occasionally, Ewing’s sarcoma may occur in soft tissues, not bone. This is called “non-osseous Ewing’s sarcoma.” Ewing’s sarcoma is a rare malignancy, which usually occurs in children and young adults less than age 30. It is not known what causes the tumor but it is not hereditary nor is it contagious. Nothing you have done or not done is responsible for your child’s tumor.

What are the symptoms of Ewing’s sarcoma?

Symptoms are often present for months before a diagnosis is made. Pain, tenderness, and swelling are the usual presenting symptoms. It may take awhile for the tumor to become large enough to cause symptoms and may become pushed out of the bone and into the surrounding soft tissues. Many children diagnosed with Ewing’s sarcoma may have a recent history of fever, weight loss, and not feeling well.

How is Ewing’s sarcoma diagnosed?
When Ewing’s sarcoma is suspected, a thorough history and physical examination are done. Since Ewing’s sarcoma may spread (metastasize) to other areas of the body your child may undergo many different tests to make an accurate diagnosis. The tests may include any or all of the following:

Surgical biopsy is the most accurate way of diagnosing and is done in all cases of Ewing’s sarcoma. Samples of the tumor and surrounding tissue are removed by a surgeon and then examined closely under the microscope by a pathologist.

  • X-rays. X-rays of tumor site and surrounding area look at size and extent of the tumor. X-rays of the chest and entire body (skeletal survey) look for spread of the tumor.
  • Bone scan. A small amount of radioactive dye is injected into a blood vessel and the body is scanned to detect any tumor in other bones.
  • Bone marrow aspirate and biopsy. A sample of bone marrow is taken from the hip bone to determine if tumor has spread to the bone marrow.
  • Computed tomography (CT) scan. This test produces images of the scanned body part to measure the extent of the tumor at the primary or original site and to look for spread of tumor in other areas of the body.
  • Magnetic resonance imaging (MRI). A computerized magnetic scan of body tissues can help determine the extent of the tumor.
  • Lumbar puncture (spinal tap). This is done to determine if the tumor has spread to the fluid surrounding the spinal cord.
  • Blood tests. Blood taken from a blood vessel is used to perform blood tests like a complete blood count (CBC) and other blood chemistries.

An important part of diagnosing and effectively treating your child is an accurate staging diagnosis. After all the tests are completed and the diagnosis has been made, a group of doctors including oncologists, radiologists, pathologists, and surgeons determine the stage of the disease and the most effective treatment.

The classification system used by your physician is as follows:

  • Localized. A localized tumor has not spread beyond the tissue in which it began.
  • Metastatic. A metastatic tumor has spread from the original tissue to other parts of the body. Ewing’s sarcoma can spread to the lung, other bone, and the bone marrow (the soft center of the bone). Spreading to the lymph nodes, brain and spinal cord is less common.

How is Ewing’s sarcoma treated?

Usually Ewing’s sarcoma is most effectively treated by a combination of surgery, chemotherapy and radiation therapy. Chemotherapy involves a combination of multiple drugs. A treatment plan is developed and explained to you in detail. During and after treatment, various follow-up studies such as x-rays, CT scans, MRI and blood tests are done to closely monitor your child’s disease status.

What research is underway?

Current clinical trials are evaluating anti-angiogenic drugs used in combination with chemotherapy for treating metastatic Ewing’s sarcoma. Anti-angiogenic drugs interfere with the tumor’s ability to make blood vessels and therefore interfere with the growth of the tumor. Other clinical trials are evaluating intensive dose chemotherapy and evaluating the use of stem cell transplants for treating Ewing’s sarcoma.

About treatment for Ewing’s sarcoma at Children’s

Children’s cancer and blood disorders program consistently achieves treatment results ranking it as one of the top 10 programs in the U.S. Children’s treats the majority of children with cancer and blood disorders in Minnesota and provides patients with access to a variety of clinical trials offering the latest new treatments. Through our renowned solid tumors program, patients experience unparalleled family support, a nationally recognized pain management team, and compassionate, coordinated care.

Contact Us

If you are a family member looking for a Children’s hematologist or oncologist or wanting to schedule an appointment, please call our clinic at Children’s – Minneapolis at 612-813-5940.

If you are a health professional looking for consultation or referral information, please call Children’s Physician Access at 1-866-755-2121 (toll-free) and ask for the on-call hematologist/oncologist.