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Bone Tumor: Ewing’s Sarcoma

What is Ewing's sarcoma?

Also at times called primitive neuroectodermal tumor, Ewing's sarcoma is one of the Ewing's family of tumors. It is one type of bone tumor , although it can also arise in soft tissues.  These are rare diseases in which cancer (malignant) cells are found in bone and soft tissues. The most common areas in which Ewing's sarcoma of the bone occurs are the extremities, pelvis and chest. The most common sites in which extraosseous (outside of bone) Ewing's sarcoma occurs are the trunk and extremities. Common sites of PNET occurrences are the chest, abdomen and pelvis.

Osteosarcoma is another type of bone cancer, but the cancer cells are different under the microscope than Ewing's sarcoma/primitive neuroectodermal tumor cancer cells.

If your child is diagnosed with a bone tumor, the best thing you can do for your child is get an opinion from a pediatric oncologist, who can refer you to a bone surgeon experienced in cancers.

Like most cancers, Ewing's sarcoma is best treated when it is found (diagnosed) early and is localized. If a patient has symptoms of Ewing's sarcoma (such as pain, stiffness, or tenderness in the bone) the doctor will order x-rays and other tests. If Ewing's sarcoma is suspected, referral should be made to a pediatric oncologist. The oncologist will work with and refer you to an oncologic surgeon if they also suspect Ewing's sarcoma.

The chance of recovery (prognosis) and choice of treatment depend on the location, size and stage of the cancer (how far the cancer has spread), how the cancer cells respond to the treatment, the patient's age and general health.

Stages of Ewing's sarcoma

Once this type of tumor has been diagnosed, more tests will be done to find out if cancer cells have spread to other parts of the body. This is called staging. At present, there is no formal staging system for Ewing's sarcoma. Instead, most patients are grouped depending on whether cancer is found in only one part of the body (localized disease) or whether cancer has spread from one part of the body to another (metastatic disease). Extraosseous Ewing's sarcoma is grouped using the rhabdomyosarcoma staging system (since it is a soft tissue tumor as well). Your oncologist needs to know where the cancer is located and how far the disease has spread to plan treatment. The following groups are used for Ewing's sarcoma:

  • Localized
    The cancer cells have not been shown to have spread beyond the bone in which the cancer began or are found only in the bone and nearby tissues.
  • Metastatic
    The cancer cells have spread from the bone in which the cancer began to other parts of the body. The cancer most often spreads to the lung, other bones, or bone marrow (the spongy tissue inside the large bones of your body that make blood cells). Spread of cancer to the lymph nodes (small bean-shaped structures found throughout the body which produce and store infection-fighting cells), or the central nervous system (brain and spinal cord) is less common.

Recurrence of disease

Recurrent disease means that the cancer has come back (recurred) after it has been treated. It may come back in the tissues where it first started or it may come back in another part of the body.

How Ewing's sarcoma/primitive neuroectodermal tumor is treated

There are treatment for all patients with Ewing's sarcoma/primitive neuroectodermal tumor. Three kinds of treatment usually used are:

  • surgery (taking out the cancer in an operation)
  • radiation therapy (using high-dose x-rays to kill cancer cells)
  • chemotherapy (using drugs to kill cancer cells)
Surgery

Surgery may be used in certain cases to try to remove the cancer and some of the tissue around it. Surgery may also be used to remove any tumor that is left after chemotherapy or radiation therapy.

Radiation therapy

Radiation therapy uses x-rays or other high-energy rays to kill cancer cells and shrink tumors. Radiation for Ewing's sarcoma usually comes from a machine outside the body (external radiation therapy). Clinical trials are evaluating radiation given inside the body during surgery (intraoperative radiation therapy).

Chemotherapy

Chemotherapy uses drugs to kill cancer cells. Chemotherapy may be taken by pill, or it may be put into the body by a needle in a vein or muscle. Chemotherapy is called a systemic treatment because the drug enters the blood stream, travels through the body, and can kill cancer cells throughout the body. When more than one drug is given to kill tumor cells, the treatment is called combination chemotherapy. For treating Ewing's sarcoma, surgery is often used to remove the primary tumor after the patient has received pre-surgical chemotherapy. Rarely, radiation therapy is given before surgery. Chemotherapy and radiation therapy are then given after surgery to kill any cancer cells that remain in the body.

Treatment by stage and clinical trial

Treatment for Ewing's sarcoma/primitive neuroectodermal tumor depends on where the cancer is located, how far the cancer has spread, the stage of the disease, the age and general health of the patient.

A patient may receive treatment that is considered standard based on its effectiveness in patients in past clinical trials, or the patient and family may choose to go into a clinical trial. Clinical trials are designed to compare potentially better therapy with that therapy which is considered standard of care in hopes of finding better ways to treat cancer patients. Clinical trials for Ewing's sarcoma/primitive neuroectodermal tumor are going on in many parts of the country. The good news is that Children's Oncology Group clinical trials are closely overseen by a data monitoring committee, so that in real time, if one treatment is found to be better than another, the treating physicians are notified immediately, and your child's treatment will be changed right away if necessary.

Localized Ewing's sarcoma/primitive neuroectodermal tumor

Treatment for localized Ewing's sarcoma/primitive neuroectodermal tumor requires multimodal therapy, as it is known that most patients with localized disease have occult (hidden, microscopic) distant spread of tumor. Treatment for this kind of tumor may be one of the following:

  1. Combination chemotherapy followed by surgery with or without radiation therapy followed by more chemotherapy.
  2. A clinical trial of chemotherapy followed by radiation therapy.
  3. A clinical trial of intensified chemotherapy
  4. A clinical trial of post-surgical chemotherapy with or without stem cell reconstitution.

Metastatic Ewing's sarcoma/primitive neuroectodermal tumor

Treatment may be one of the following:

  1. Combination chemotherapy, followed by radiation therapy and /or surgery
  2. High dose chemotherapy with or without radiation therapy plus stem cell rescue
  3. A clinical trial of intensive chemotherapy utilizing combination chemotherapy.

Recurrent Ewing's sarcoma/primitive neuroectodermal tumor

Treatment for recurrent Ewing's sarcoma depends on where the cancer recurred, how the cancer was treated before, and individual patient factors. Chemotherapy may be used. Radiation treatment may be given to reduce symptoms. Surgery may be used to remove the areas that have spread to the lungs or other organs. Clinical trials are testing new treatments.

Cleveland Clinic Children's

Here at Cleveland Clinic Children's, we specialize in the care of children and young adults with cancer. We use a team approach that cares for all members of the family, recognizing that this is a very difficult time for all of them. Our goal is to have our patients get through therapy with as normal a life as possible, and to emerge from therapy knowledgeable about how to maintain their health.

Should you have more questions about the child or young adult in your life with cancer, please contact Cleveland Clinic Children's Cancer Answer line at 216.444.5517, option 1, or 800.223.2273, extension 4-5517, option 1. Your question will be forwarded to the Chair of the Pediatric Oncology Department.

We wish you and your child well.

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