Description

What is the Ewing's family of tumors?

The Ewing's family of tumors include: Ewing's tumor of bone; extraosseus Ewing's (tumor growing outside of the bone); primitive neuroectodermal tumor (PNET), also known as peripheral neuroepithelioma; and Askin's tumor (PNET of the chest wall). These tumors are rare diseases in which cancer (malignant) cells are found in the bone and soft tissues. Ewing's family of tumors most frequently occurs in teenagers.

If a patient has symptoms (such as pain, stiffness, or tenderness in the bone) the doctor may order x-rays and other tests. The doctor may also cut out a piece of tissue from the affected area. This is called a biopsy. The tissue will be looked at under a microscope to see if there are any cancer cells. This test may be done in the hospital.

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 react to the treatment, and the patient's age and general health. 

Stage Explanation

Stages of the Ewing's family of tumors

Once one of the Ewing's family of tumors has been found, 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 the Ewing's family of tumors. 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 has been grouped using the rhabdomyosarcoma staging system because they are both soft tissue tumors. Your doctor needs to know where the cancer is located and how far the disease has spread to plan treatment. The following groups are used for the Ewing's family of tumors.

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, and bone marrow (the spongy tissue inside of the large bones of your body that makes red blood cells). Spread of cancer to the lymph nodes (small bean-shaped structures found throughout your body which produce and store infection-fighting cells) or the central nervous system (brain and spinal cord) is less common.

Recurrent

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. 

Treatment Option Overview

How the Ewing's family of tumors are treated

It is important for patients to be evaluated by several specialists as early as possible so that treatment may be coordinated effectively from the beginning. These specialists may include: a radiologist, chemotherapist, pathologist, surgeon, or orthopedic oncologist and a radiation oncologist. Before treatment decisions are made patients will probably be required to undergo several diagnostic tests including tissue sampling, x-rays, magnetic resonance imaging (MRI) scans, and computed tomography (CT) scans.

There are treatments for all patients with one of the Ewing's family of tumors. Three kinds of treatment are used:

•  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 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 uses x-rays or other high-energy rays to kill cancer cells and shrink tumors. Radiation for the Ewing's family of tumors 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 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 the Ewing's family of tumors, surgery or radiation is often used to remove the local tumor and chemotherapy is then given to kill any cancer cells that remain in the body.

A supplement to the treatment options listed above is myeloablative therapy with stem cell support. Myeloablative therapy is a very intense regimen of chemotherapy designed to destroy all cells that divide rapidly. These cells include some blood cells and hair cells, as well as malignant (cancer) cells. Stem cells are self-renewing cells that create all of the other various types of blood cells. Stem cell support involves enriching the stem cells to increase the number of these important cells circulating in the blood after the chemotherapy has been given to kill the remaining tumor cells.

Treatment for the Ewing's family of tumors depends on where the cancer is located, how far the cancer has spread, the stage of the disease, and the age and general health of the patient.

A patient may receive treatment that is considered standard based on its effectiveness in a number of patients in past studies, or may choose to go into a clinical trial. Not all patients are cured with standard therapy and some standard treatments may have more side effects than are desired. For these reasons, clinical trials are designed to find better ways to treat cancer patients and are based on the most up-to-date information. Clinical trials for the Ewing's family of tumors are ongoing in many parts of the country. If you want more information, call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237); TTY at 1-800-332-8615. 

Localized Tumors of the Ewing's Family

Treatment for localized tumors of the Ewing's family may be one of the following:

1. A clinical trial of chemotherapy followed by radiation therapy

2. Combination chemotherapy followed by surgery with or without radiation therapy

3. A clinical trial of intensified chemotherapy

4. A randomized trial of post-surgical chemotherapy with or without stem cell transplant

Metastatic Tumors of the Ewing's Family 

Treatment for metastatic tumors of the Ewing's family 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 additional stem cell support

3. A clinical trial of intensive chemotherapy with multiple chemotherapy drug combinations

Recurrent Tumors of the Ewing's Family

Treatment depends on where the cancer recurred, how the cancer was treated before, as well as individual patient factors. Chemotherapy may be used for patients who did not receive previous chemotherapy. Radiation treatment may be given to reduce symptoms. Surgery may be used to remove tumors that have spread to the lungs or other organs. Clinical trials are testing new treatments. 

Published by National Cancer Institute on 9/10/2002.