CancerNet from the National Cancer Institute
Information from PDQ for Patients
What is PDQ?
PDQ is a computer system that provides up-to-date information on cancer and its
prevention, detection, treatment, and supportive care. PDQ is a service of the
National Cancer Institute (NCI) for people with cancer and their families and
for doctors, nurses, and other health care professionals.
To ensure that it remains current, the information in PDQ is reviewed and
updated each month by experts in the fields of cancer treatment, prevention,
screening, and supportive care. PDQ also provides information about research
on new treatments (clinical trials), doctors who treat cancer, and hospitals
with cancer programs. The treatment information in this summary is based on
information in the PDQ summary for health professionals on this cancer.
How to use PDQ
Cancer in children is rare. The majority of children with cancer are treated
at cancer centers with special facilities to treat childhood cancers. There
are organized groups of doctors and other health care professionals who work
together by doing clinical trials to improve treatments for children with
PDQ can be used to learn more about current treatment of different kinds of
cancer. You may find it helpful to discuss this information with your child's
doctor, who knows your child and has the facts about your child's disease. PDQ
can also provide the names of additional health care professionals and
hospitals that specialize in treating children who have cancer.
Before your child begins treatment, you may want to consider entering your
child in a clinical trial. PDQ can be used to learn more about the trials. A
clinical trial is a research study that attempts to improve current treatments
or find new treatments for people with cancer. Clinical trials are based on
past studies and information discovered in the laboratory. Each trial answers
specific scientific questions in order to find new and better ways to help
people with cancer. During clinical trials, information is collected about new
treatments, their risks, and how well they do or do not work. When a clinical
trial shows that a new treatment is better than the treatment currently used as
"standard" treatment, the new treatment may become the "standard" treatment.
Children who are treated in clinical trials have the advantage of getting the
best available therapy. In the United States, about two thirds of children
with cancer are treated in a clinical trial at some point in their illness.
Listings of current clinical trials are available on PDQ. In the United
States, there are two major groups (called cooperative groups) that organize
clinical trials for childhood cancers: the Childrens Cancer Group (CCG) and the
Pediatric Oncology Group (POG). Doctors who belong to these groups or who take
part in other clinical trials are listed in PDQ.
To learn more about cancer and how it is treated or to learn more about
clinical trials for your child's kind of cancer,
- call the National Cancer
Institute's Cancer Information Service. The number is 1-800-4-CANCER
(1-800-422-6237) The call is free and a trained
information specialist will be available to answer your cancer-related
PDQ is updated whenever there is new information. Check with the Cancer
Information Service to be sure that you have the most up-to-date information.
What is Ewing's sarcoma/primitive neuroectodermal tumor?
Ewing's sarcoma/primitive neuroectodermal tumor is a rare disease in which
cancer (malignant) cells are found in the bone. The most common areas in which
it occurs are the pelvis, the thigh bone (femur), the upper arm bone (humerus),
and the ribs. Ewing's sarcoma/primitive neuroectodermal tumor most frequently
occurs in teenagers.
Osteosarcoma is another kind of bone cancer, but the cancer cells look
different under a microscope than Ewing's sarcoma/primitive neuroectodermal
tumor cancer cells. If you want information on osteosarcoma, refer to the PDQ
patient information statement on osteosarcoma.
Like most cancers, Ewing's sarcoma/primitive neuroectodermal tumor is best
treated when it is found (diagnosed) early. 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
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
Stages of Ewing's sarcoma/primitive neuroectodermal tumor
Once Ewing's sarcoma/primitive neuroectodermal tumor 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 Ewing's sarcoma/primitive neuroectodermal tumor. 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). 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 Ewing's sarcoma/primitive neuroectodermal tumor:
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.
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 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 treatments for all patients with Ewing's sarcoma/primitive
neuroectodermal tumor. 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.
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)
Radiation therapy uses x-rays or other high-energy rays to kill cancer cells
and shrink tumors. Radiation for Ewing's sarcoma/primitive neuroectodermal
tumor 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 Ewing's sarcoma/primitive
neuroectodermal tumor, surgery or radiation is often used to remove the local
tumor and chemotherapy is then given to kill any cancer cells that remain in
Treatment by stage
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,
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
Ewing's sarcoma/primitive neuroectodermal tumor are going on 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)
Treatment for localized Ewing's sarcoma/primitive neuroectodermal tumor depends
on where the cancer is found in the body.
If the cancer is in the bone below the elbow or knee or in the jaw, skull,
face, shoulder blade, collar bone, or segments of the spinal column, treatment
may be one of the following:
Clinical trials are also evaluating new doses and combinations of chemotherapy.
1. Combination chemotherapy
2. Surgery and combination chemotherapy.
3. Radiation therapy and combination chemotherapy.
4. A clinical trial of chemotherapy and new ways of giving radiation therapy.
5. A clinical trial of chemotherapy followed by surgery, with or without
Treatment may be one of the following:
Clinical trials are evaluating new doses and combinations of chemotherapy with
or without radiation treatment.
1. Combination chemotherapy.
2. Radiation therapy plus combination chemotherapy.
3. Combination chemotherapy plus surgery to remove cancer that has spread to
Treatment depends on where the cancer recurred, how the cancer was treated
before, and individual patient factors. Radiation treatment may be given to
reduce symptoms. Clinical trials are testing new treatments.
TO LEARN MORE..... CALL 1-800-4-CANCER