Ewing Family of Tumors Treatment (PDQ®)
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Description
Note: The health professional version of this summary was extensively revised in July 2007. The patient version of this summary will reflect these changes shortly.
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What is the Ewing family of tumors?
The Ewing family of tumors include: Ewing tumor of bone; extraosseous
Ewing (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 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.
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Stage Explanation
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Stages of the Ewing family of tumors
Once one of the Ewing 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 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 has been grouped using the rhabdomyosarcoma
staging system because they are both soft tissue tumors. (Refer to the PDQ
summary on Rhabdomyosarcoma Treatment for more information.) 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 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.
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Treatment Option Overview
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How the Ewing 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 family of tumors.
Three kinds of treatment are used:
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 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. Treatment for the Ewing family of tumors may include surgery or radiation to remove or shrink the tumor as much as possible, followed by chemotherapy 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 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 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.
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Localized Tumors of the Ewing Family
Treatment for localized tumors of the Ewing family may be one of the
following:
Check for clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with localized Ewing sarcoma/peripheral primitive neuroectodermal tumor.
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Metastatic Tumors of the Ewing Family
Treatment for metastatic tumors of the Ewing family may be one of the
following:
Check for clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with metastatic Ewing sarcoma/peripheral primitive neuroectodermal tumor.
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Recurrent Tumors of the Ewing 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.
Check for clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with recurrent Ewing sarcoma/peripheral primitive neuroectodermal tumor.
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Changes to This Summary (08/06/2007)
The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.
Editorial changes were made to this summary to match those made to the health professional version. Extensive content revisions to match those made to the health professional version will follow shortly.
This information is provided from the PDQ® database in collaboration with the NCI.
