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National Cancer Institute

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Childhood soft tissue sarcoma


Table of Contents

OVERVIEW OF PDQ
What is PDQ?
How to use PDQ
DESCRIPTION
What is childhood soft tissue sarcoma?
STAGE EXPLANATION
Stages of childhood soft tissue sarcoma
Desmoid tumor
Nonmetastatic childhood soft tissue sarcoma
Metastatic childhood soft tissue sarcoma
Recurrent soft tissue sarcoma
TREATMENT OPTION OVERVIEW
How childhood soft tissue sarcoma is treated
Treatment by stage
NONMETASTATIC CHILDHOOD SOFT TISSUE SARCOMA
METASTATIC CHILDHOOD SOFT TISSUE SARCOMA
RECURRENT CHILDHOOD SOFT TISSUE SARCOMA
TO LEARN MORE

OVERVIEW OF PDQ


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 and adolescents 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 cancer.

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); TTY at 1-800-332-8615. The call is free and a trained information specialist will be available to answer your cancer-related questions.

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.


DESCRIPTION


What is childhood soft tissue sarcoma?

Childhood soft tissue sarcoma is a disease in which cancer (malignant) cells begin growing in soft tissue somewhere in the body. The soft tissues include muscles, tendons (bands of fiber that connect muscles to bones), fibrous tissues, fat, blood vessels, nerves, and synovial tissues (tissues around joints). Soft tissues connect, support, and surround other body parts and organs.

Soft tissue sarcomas are rare in infants and children. If a patient has symptoms of a soft tissue sarcoma, the doctor may order x-rays and other tests. The doctor may also cut out a small piece of tissue and have it looked at under the microscope to see if there are any cancer cells. This is called a biopsy.

There are many different kinds of soft tissue sarcoma, depending on the soft tissue where the cancer begins. Rhabdomyosarcoma is the most common type of childhood soft tissue sarcoma. It begins in muscles around the bone and can be found anywhere in the body. If you want information on rhabdomyosarcoma, refer to the PDQ patient information statement on childhood rhabdomyosarcoma. PDQ also contains a patient information statement on adult soft tissue sarcoma.

The following types of soft tissue sarcoma in children are covered in this statement:

The chance of recovery (prognosis) and choice of treatment depend on the type, location, and stage of the tumor and the age, size, stage of development, and general health of the patient.


STAGE EXPLANATION


Stages of childhood soft tissue sarcoma

Once childhood soft tissue sarcoma is found, more tests will be done to find out if the cancer cells have spread to other parts of the body. This is called staging. Your doctor needs to know the stage of the cancer to plan treatment.

There are several staging systems for childhood soft tissue sarcoma. There is not one that applies to all types of this cancer. The treatment options in this statement are based on whether the cancer has spread or the amount of tumor left after surgery.


Desmoid tumor

Desmoid tumors are not cancer. Desmoid tumors may be confused with a type of fibrosarcoma.


Nonmetastatic childhood soft tissue sarcoma

The cancer is found only in the area where it started and has not spread to other parts of the body.


Metastatic childhood soft tissue sarcoma

The cancer has spread from where it started to other parts of the body.


Recurrent soft tissue sarcoma

The cancer has come back (recurred) after it has been treated. It may come back in the area where it started or in another part of the body.


TREATMENT OPTION OVERVIEW


How childhood soft tissue sarcoma is treated

There are treatments for all patients with childhood soft tissue sarcoma. Three types of treatment are used, most often in combination with each other:

surgery (taking out the cancer in an operation)
radiation therapy (using high-dose x-rays or other high-energy rays to kill
cancer cells)
chemotherapy (using drugs to kill cancer cells).

Surgery is a common treatment for soft tissue sarcoma. Depending on where the cancer is, the surgeon may take out as much of the cancer as possible, along with some of the normal tissue around it.

Radiation therapy uses high-energy x-rays to kill cancer cells and shrink tumors. Radiation may come from a machine outside the body (external radiation therapy) or from putting materials that produce radiation (radioisotopes) through thin plastic tubes in the area where the cancer cells are found (internal radiation therapy). Clinical trials are testing radiation given in several small doses per day (hyperfractionated radiation therapy).

Chemotherapy uses drugs to kill cancer cells. Chemotherapy may be taken by mouth in the form of a 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 drugs enter the bloodstream, travel through the body, and can kill cancer cells throughout the body.


Treatment by stage

Treatment for soft tissue sarcoma depends on where the cancer is, how far it has spread, and what the cancer cells look like under the microscope.

The patient may receive treatment that is considered standard based on its effectiveness in a number of patients in past studies, or the doctor may recommend the patient enter 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 test new treatments and to find better ways to treat cancer patients. A large cooperative group clinical trial comparing new treatments with standard treatments is going on in most parts of the country for all stages of soft tissue sarcoma. 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.


NONMETASTATIC CHILDHOOD SOFT TISSUE SARCOMA

Treatment depends on the type of soft tissue sarcoma.

If your child has fibrosarcoma (older child or teenager), neurofibrosarcoma, liposarcoma, synovial sarcoma, hemangiopericytoma (older than 1 year of age), malignant fibrous histiocytoma, or leiomyosarcoma, treatment may be one of the following:

1. Surgery to remove all of the cancer. Sometimes a second operation must be done to be sure that all the tumor has been removed.

2. Surgery to remove the cancer followed by radiation therapy (if small amounts of tumor remain after surgery).

3. Surgery to remove the cancer followed by radiation therapy and chemotherapy (if larger amounts of tumor remain after surgery).

Clinical trials are evaluating new ways to combine chemotherapy and radiation therapy with surgery.

If your child is younger and has fibrosarcoma or is under 1 year old and has hemangiopericytoma, treatment may be one of the following:

1. Surgery to remove all of the cancer. Sometimes a second operation must be done to be sure that all the tumor has been removed.

2. Chemotherapy and radiation therapy if the tumor cannot be removed by surgery.

If your child has desmoid tumor, treatment will probably be surgery to remove the tumor. If any tumor remains after surgery, your child will probably receive radiation therapy or chemotherapy.

If your child has alveolar soft part sarcoma, treatment may be one of the following:

1. Surgery to remove all of the cancer.

2. Surgery followed by radiation therapy (if the cancer is not completely removed during surgery).

3. Surgery followed by chemotherapy. This treatment is still being evaluated in clinical trials.

Clinical trials are evaluating new ways to combine chemotherapy and radiation therapy with surgery.


METASTATIC CHILDHOOD SOFT TISSUE SARCOMA

Desmoplastic round-cell tumors are abdominal tumors that spread rapidly and usually occur in males in their teens and twenties. Treatment will probably be surgery, radiation, and chemotherapy. Clinical trials are evaluating new combinations of chemotherapy.


RECURRENT CHILDHOOD SOFT TISSUE SARCOMA

Treatment for recurrent childhood soft tissue sarcoma depends on what treatment your child received before, the part of the body where the cancer has come back, and your child's general condition. You may wish to have your child take part in a clinical trial.


TO LEARN MORE

TO LEARN MORE..... CALL 1-800-4-CANCER

To learn more about childhood soft tissue sarcoma, call the National Cancer Institute's Cancer Information Service at 1-800-4-CANCER (1-800-422-6237); TTY at 1-800-332-8615. By dialing this toll-free number, you can speak with someone who can answer your questions.

The Cancer Information Service can also send you booklets. The following booklets on childhood cancer may be helpful to you:

Young People with Cancer: A Handbook for Parents
Talking with Your Child About Cancer
Managing Your Child's Eating Problems During Cancer Treatment
When Someone in Your Family Has Cancer

The following general booklets on questions related to cancer may also be helpful:

Taking Time: Support for People with Cancer and the People Who Care About
Them
What Are Clinical Trials All About?
Chemotherapy and You: A Guide to Self-Help During Treatment
Radiation Therapy and You: A Guide to Self-Help During Treatment
What You Need To Know About Cancer

There are many other places you can get material about cancer treatment and services to help you. You can check the social service office at your hospital for local and national agencies that help with your finances, getting to and from treatment, care at home, and dealing with your problems.

You can also write to the National Cancer Institute at this address:

National Cancer Institute
Office of Cancer Communications
31 Center Drive, MSC 2580
Bethesda, MD 20892-2580

Date Last Modified: 09/1999


If you want to know more about cancer and how it is treated, or if you wish to know about clinical trials for your type of cancer, you can call the NCI's Cancer Information Service at 1-800-422-6237, toll free. A trained information specialist can talk with you and answer your questions.


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