Childhood Soft Tissue Sarcoma
What is childhood soft tissue sarcoma?
Soft tissue sarcomas are rare in children and adolescents. If a patient has
There are many different kinds of soft tissue sarcoma, depending on the soft tissue where the cancer begins.
Soft tissue sarcomas may develop in any part of the body, but in young patients, they are most commonly found in the trunk, arms, and legs. The first symptom may be a solid mass or lump. If the mass interferes with a function of the body, it may cause other symptoms. Soft tissue sarcoma rarely causes
Soft tissue sarcoma is more likely to develop in people who have specific
Soft tissue sarcomas are classified according to the type of soft tissue they resemble. The types of soft tissue sarcoma include:
Tumors of fibrous (connective) tissue:
desmoid tumor fibrosarcoma
malignant fibrous histiocytoma
Fat tissue tumors:
Smooth muscle tumors:
Blood and lymph vessel tumors:
Peripheral nervous system tumors:
schwannoma(malignant peripheral nerve sheath tumor)
Bone and cartilage tumors:
Tumors with more than one type of tissue:
malignant Triton tumor
Tumors of unknown origin:
alveolar soft part sarcoma
clear cellsarcoma (malignant melanomaof soft parts [MMSP]) synovial sarcoma desmoplastic small round cell tumor
The chance of
Stages of childhood soft tissue sarcoma
There are several staging systems for childhood
Nonmetastatic childhood soft tissue sarcoma
The cancer has been partly or completely removed in surgery 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 (
Treatment Option Overview
How childhood soft tissue sarcoma is treated
There are treatments for all patients with childhood
Surgery(taking out the cancerin an operation). Radiation therapy(using high- dose x-raysor other high-energy rays to kill cancer cells). Chemotherapy(using drugsto kill cancer cells).
Surgery is the
Radiation therapy uses high-energy rays to kill cancer cells and shrink
Chemotherapy uses drugs to kill cancer cells. Chemotherapy may be given before or after surgery. 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
Some cancer treatments cause
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 a 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 that the patient enter a clinical trial. Not all patients are
Nonmetastatic Childhood Soft Tissue Sarcoma
Treatment depends on the type of
If your infant or young child has
Surgeryto remove all of the cancer.Sometimes a second operation must be done to be sure that all the tumorhas been removed, or if the tumor comes back following treatment.
Surgery to remove the cancer followed by
radiation therapy(if a second surgery is not possible). Chemotherapyto reduce the tumor size, followed by surgery.
If your child has desmoid tumor, treatment may be one of the following:
Surgery to remove all of the cancer.
Radiation therapy given before or after surgery, nonsteroidal antiinflammatory agents, antiestrogens, and chemotherapy (if complete removal of the tumor is not possible).
Patients may undergo close
monitoringwhen no other treatment alternatives are available and the tumor does not place any vital organsin danger (if complete removal of the tumor is not possible or if the tumor comes back following treatment).
Clinical trials are evaluating the effectiveness of
If your older child or adolescent has fibrosarcoma or has hemangiopericytoma, or if your child has
Surgery to remove all of the cancer. Sometimes a second operation must be done to be sure that all the tumor has been removed.
Surgery and radiation therapy (if the tumor cannot be completely removed by surgery).
A clinical trial of surgery followed by chemotherapy, for synovial sarcoma.
The effectiveness of
If your child has alveolar soft part sarcoma, treatment may be one of the following:
Surgery to remove all of the cancer.
Surgery followed by radiation therapy (if the cancer is not completely removed during surgery).
If your child has
If your child has
If your infant has hemangioendothelioma, treatment may be one of the following:
Surgery for tumors with
symptoms. Liver transplant.
If your child has a vascular tumor, treatment will probably be surgery.
If your child has undifferentiated sarcoma, which is similar in some ways to
Metastatic Childhood Soft Tissue Sarcoma
Children who have
Recurrent Childhood Soft Tissue Sarcoma
Surgeryto remove the cancer.
Surgery followed by
radiation therapy(if the patient has not yet received radiation therapy). Amputationof sarcomasof the arm or leg in patients who have previously received radiation therapy.
Changes to This Summary (10/18/2006)
Editorial changes were made to this summary.
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PDQ is a comprehensive cancer database available on NCI's Web site.
PDQ is the National Cancer Institute's (NCI's) comprehensive cancer information database. Most of the information contained in PDQ is available online at NCI's Web site. PDQ is provided as a service of the NCI. The NCI is part of the National Institutes of Health, the federal government's focal point for biomedical research.
PDQ contains cancer information summaries.
The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries are available in two versions. The health professional versions provide detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions provide current and accurate cancer information.
The PDQ cancer information summaries are developed by cancer experts and reviewed regularly.
Editorial Boards made up of experts in oncology and related specialties are responsible for writing and maintaining the cancer information summaries. The summaries are reviewed regularly and changes are made as new information becomes available. The date on each summary ("Date Last Modified") indicates the time of the most recent change.
PDQ also contains information on clinical trials.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about new treatments, the risks involved, and how well they do or do not work. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." 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 clinical trials are included in PDQ and are available online at NCI's Web site. Descriptions of the trials are available in health professional and patient versions. For additional help in locating a childhood cancer clinical trial, call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237), TTY at 1-800-332-8615.
The PDQ database contains listings of groups specializing in clinical trials.
The Children's Oncology Group (COG) is the major group that organizes clinical trials for childhood cancers in the United States. Information about contacting COG is available on the NCI Web site or from the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237), TTY at 1-800-332-8615.
The PDQ database contains listings of cancer health professionals and hospitals with cancer programs.
Because cancer in children and adolescents is rare, the majority of children with cancer are treated by health professionals specializing in childhood cancers, at hospitals or cancer centers with special facilities to treat them. The PDQ database contains listings of health professionals who specialize in childhood cancer and listings of hospitals with cancer programs. For help locating childhood cancer health professionals or a hospital with cancer programs, call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237), TTY at 1-800-332-8615.