Home > Health & Wellness > Health Library > Childhood Soft Tissue Sarcoma Treatment (PDQ®): Treatment - Patient Information [NCI]
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Childhood soft tissue sarcoma is a disease in which malignant (cancer) cells form in soft tissues of the body.
Soft tissues of the body connect, support, and surround other body parts and organs. The soft tissues include the following:
Soft tissue sarcoma may be found anywhere in the body. In children, the tumors form most often in the arms, legs, or trunk (chest and abdomen).
There are many different types of soft tissue sarcomas.
The cells of each type of sarcoma look different under a microscope. The soft tissue tumors are grouped based on the type of soft tissue cell where they first formed.
This summary is about the following types of soft tissue sarcoma:
Fat tissue tumors
Bone and cartilage tumors
Bone and cartilage tumors are a mix of bone cells and cartilage cells. Bone and cartilage tumors include the following types:
Fibrous (connective) tissue tumors
Fibrous (connective) tissue tumors include the following types:
Desmoid tumors sometimes occur in children with changes in the adenomatous polyposis coli (APC) gene. Changes in this gene cause familial adenomatous polyposis (FAP). FAP is an inherited condition in which many polyps (growths on mucous membranes) form on the inside walls of the colon and rectum. Genetic counseling (a discussion with a trained professional about inherited diseases and a possible need for gene testing) may be needed.
There are two types of fibrosarcoma in children and teenagers:
Skeletal muscle tumors
Skeletal muscle is attached to bones and helps the body move.
Smooth muscle tumors
Smooth muscle lines the inside of blood vessels and hollow internal organs such as the stomach, intestines, bladder, and uterus.
So-called fibrohistiocytic tumors
So-called fibrohistiocytic tumors include the following types:
Peripheral nervous system tumors
Tumors of unknown origin
Tumors of unknown origin (the place where the tumor first formed is not known) include the following types:
Blood vessel tumors
Blood vessel tumors include the following types:
See the following PDQ summaries for information about types of soft tissue sarcoma not included in this summary:
Soft tissue sarcoma occurs in children and adults.
Soft tissue sarcoma in children may respond differently to treatment, and may have a better prognosis than soft tissue sarcoma in adults. (See the PDQ summary on Adult Soft Tissue Sarcoma Treatment for more information on treatment in adults.)
Having certain diseases and inherited disorders can increase the risk of childhood soft tissue sarcoma.
Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your child's doctor if you think your child may be at risk.
Risk factors for childhood soft tissue sarcoma include having the following inherited disorders:
Other risk factors include the following:
The most common sign of childhood soft tissue sarcoma is a painless lump or swelling in soft tissues of the body.
A sarcoma may appear as a painless lump under the skin, often on an arm, a leg, or the trunk. There may be no other symptoms at first. As the sarcoma grows and presses on nearby organs, nerves, muscles, or blood vessels, it may cause symptoms, such as pain or weakness.
Other conditions may cause the same symptoms as soft tissue sarcoma. Check with your child's doctor if you see any of these problems in your child.
Diagnostic tests and a biopsy are used to detect (find) and diagnose childhood soft tissue sarcoma.
The following tests and procedures may be used:
If these tests show there may be a soft tissue sarcoma, a biopsy is done. One of the following types of biopsies may be used:
In order to plan the best treatment, the sample of tissue removed during the biopsy must be large enough to find out the type of soft tissue sarcoma and do other laboratory tests. Tissue samples will be taken from the primary tumor, lymph nodes, and other areas that may have a tumor. A pathologist views the tissue under a microscope to look for cancer cells and to find out the type and grade of the tumor. The grade of a tumor depends on how abnormal the cancer cells look under a microscope and how quickly the cells are dividing. High-grade and mid-grade tumors usually grow and spread more quickly than low-grade tumors.
Because soft tissue sarcoma can be hard to diagnose, parents should ask to have the tissue sample checked by a pathologist who has experience in diagnosing soft tissue sarcoma.
One or more of the following laboratory tests may be done to study the tissue samples:
Certain factors affect prognosis (chance of recovery) and treatment options.
The prognosis (chance of recovery) and treatment options depend on the following:
After childhood soft tissue sarcoma has been diagnosed, tests are done to find out if cancer cells have spread to other parts of the body.
The process used to find out if cancer has spread within the soft tissue or to other parts of the body is called staging. There is no standard staging system for childhood soft tissue sarcoma. Two methods that are commonly used for staging are based on the following:
It is important to know the stage in order to plan treatment.
The following tests and procedures may be used in the staging process:
The results of the sentinel lymph node biopsy and CT scan are viewed together with the results of the diagnostic tests and initial surgery to find out the stage of the soft tissue sarcoma.
There are three ways that cancer spreads in the body.
Cancer can spread through tissue, the lymph system, and the blood:
Cancer may spread from where it began to other parts of the body.
When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood.
The metastatic tumor is the same type of cancer as the primary tumor. For example, if soft tissue sarcoma spreads to the lung, the cancer cells in the lung are soft tissue sarcoma cells. The disease is metastatic soft tissue sarcoma, not lung cancer.
One method used to stage childhood soft tissue sarcoma is based on how much cancer remains after surgery to remove the tumor and whether the cancer has spread:
Nonmetastatic childhood soft tissue sarcoma
In nonmetastatic childhood soft tissue sarcoma, the cancer has been partly or completely removed by surgery and has not spread to other parts of the body.
Metastatic childhood soft tissue sarcoma
Another method used to stage childhood soft tissue sarcoma is based on the size of the tumor and whether cancer has spread to lymph nodes or other parts of the body.
This staging system is based on the following:
Pea, peanut, walnut, and lime show tumor sizes.
Stage I is divided into stages IA and IB:
Stage II is divided into stage IIA and stage IIB:
In stage III, the tumor is either:
In stage IV, cancer has spread to distant parts of the body such as the lungs. The tumor is any grade or size, and may have spread to nearby lymph nodes.
Recurrent childhood soft tissue sarcoma is cancer that has recurred (come back) after it has been treated. The cancer may come back in the same place or in other parts of the body.
Progressive childhood soft tissue sarcoma is cancer that did not respond to treatment.
There are different types of treatment for patients with childhood soft tissue sarcoma.
Different types of treatments are available for patients with childhood soft tissue sarcoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment.
Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment.
Children with childhood soft tissue sarcoma should have their treatment planned by a team of health care providers who are experts in treating cancer in children.
Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other health care providers who are experts in treating children with soft tissue sarcoma and who specialize in certain areas of medicine. These may include a pediatric surgeon with special training in the removal of soft tissue sarcomas. The following specialists may also be included:
Some cancer treatments cause side effects months or years after treatment has ended.
Side effects from cancer treatment that begin during or after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include:
Some late effects may be treated or controlled. It is important to talk with your child's doctors about the effects cancer treatment can have on your child. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information.)
Seven types of standard treatment are used:
Surgery to completely remove the soft tissue sarcoma is done when possible. If the tumor is very large, radiation therapy or chemotherapy may be given first, to make the tumor smaller and decrease the amount of tissue that needs to be removed during surgery.
The following types of surgery may be used:
A second surgery may be needed to:
If cancer is in the liver, a liver transplant may be done (the liver is removed and replaced with a healthy one from a donor).
Even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given radiation therapy or chemotherapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy.
Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. In general, radiation therapy is given when the tumor is not completely removed by surgery or is likely to grow and spread quickly.
There are two types of radiation therapy:
The way the radiation therapy is given depends on the type and stage of the cancer being treated.
Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is the use of more than one anticancer drug. The way the chemotherapy is given depends on the type and stage of the cancer being treated.
Most types of soft tissue sarcoma do not respond to treatment with chemotherapy.
See Drugs Approved for Adult and Childhood Soft Tissue Sarcoma for more information.
Watchful waiting is closely monitoring a patient's condition without giving any treatment until signs or symptoms appear or change. Watchful waiting may be done when:
Hormone therapy is a cancer treatment that removes hormones or blocks their action and stops cancer cells from growing. Hormones are substances made by glands in the body and circulated in the bloodstream. Some hormones can cause certain cancers to grow. If tests show that the cancer cells have places where hormones can attach (receptors), drugs, surgery, or radiation therapy is used to reduce the production of hormones or block them from working. Antiestrogens (drugs that block estrogen) may be used to treat childhood soft tissue sarcoma.
Nonsteroidal anti-inflammatory drugs
Nonsteroidal anti-inflammatory drugs (NSAIDs) are drugs (such as aspirin, ibuprofen, and naproxen) that are commonly used to decrease fever, swelling, pain, and redness. In the treatment of soft tissue sarcomas, an NSAID called sulindac may be used to help block the growth of cancer cells.
Targeted therapy is a type of treatment that uses drugs or other substances to find and attack specific cancer cells without harming normal cells. Imatinib is a type of targeted therapy called a tyrosine kinase inhibitor. It finds and blocks an abnormal protein on cancer cells that causes them to divide and grow. It is used in the treatment of dermatofibrosarcoma protuberans. Pazopanib is also a tyrosine kinase inhibitor used in the treatment of recurrent or progressive soft tissue sarcoma. An mTOR inhibitor is another type of targeted therapy. An mTOR inhibitor stops the protein that helps cells divide and survive. It is used to treat perivascular epithelioid cell tumors.
Other targeted therapies are being studied in clinical trials including angiogenesis inhibitors. In cancer treatment, angiogenesis inhibitors prevent the growth of new blood vessels needed for tumors to grow. They are used to treat alveolar soft part sarcoma and blood vessel tumors.
New types of treatment are being tested in clinical trials.
Information about clinical trials is available from the NCI Web site.
Patients may want to think about taking part in a clinical trial.
For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.
Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.
Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.
Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.
Follow-up tests may be needed.
Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. This is sometimes called re-staging.
Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.
Newly Diagnosed Childhood Soft Tissue Sarcoma
Fat Tissue Tumors
Treatment of liposarcoma may include the following:
Bone and Cartilage Tumors
Treatment of extraskeletal chondrosarcoma may include the following:
Treatment of extraskeletal osteosarcoma may include the following:
In children, it is not clear if chemotherapy improves the tumor's response to treatment.
Fibrous (Connective) Tissue Tumors
Treatment of desmoid tumor may include the following:
If surgery to completely remove the tumor is not possible, treatment to shrink the tumor before surgery may include the following:
Treatment of infantile fibrosarcoma (also called congenital fibrosarcoma) may include the following:
Treatment of adult-type fibrosarcoma may include the following:
Treatment of dermatofibrosarcoma protuberans may include the following:
Inflammatory myofibroblastic tumor
Treatment of inflammatory myofibroblastic tumor may include the following:
Low-grade fibromyxoid sarcoma
There is no standard treatment for low-grade fibromyxoid sarcoma.
Treatment of myxofibrosarcoma, low-grade may include the following:
Sclerosing epithelioid fibrosarcoma
Treatment of sclerosing epithelioid fibrosarcoma may include the following:
Skeletal Muscle Tumors
See the PDQ summary on Childhood Rhabdomyosarcoma Treatment.
Smooth Muscle Tumors
There is no standard treatment for leiomyosarcoma.
So-called Fibrohistiocytic Tumors
Plexiform fibrohistiocytic tumor
Treatment of plexiform fibrohistiocytic tumor may include the following:
Undifferentiated pleomorphic sarcoma
There is no standard treatment for undifferentiated pleomorphic sarcoma.
Peripheral Nervous System Tumors
Malignant peripheral nerve sheath tumor
The treatment of malignant peripheral nerve sheath tumor may include the following:
It is not clear whether giving radiation therapy or chemotherapy after surgery improves the tumor's response to treatment.
Tumors of Unknown Origin (the place where the tumor first formed is not known)
Alveolar soft part sarcoma
Treatment of alveolar soft part sarcoma may include the following:
Clear cell sarcoma of soft tissue
Treatment of clear cell sarcoma of soft tissue may include the following:
Desmoplastic small round cell tumor
There is no standard treatment for desmoplastic small round cell tumor. Treatment may include the following:
Treatment of epithelioid sarcoma may include the following:
Perivascular epithelioid cell tumors (PEComas)
Treatment of perivascular epithelioid cell tumors may include the following:
Extrarenal (extracranial) rhabdoid tumor
Treatment of extrarenal (extracranial) rhabdoid tumor may include the following:
Extraskeletal myxoid chondrosarcoma
Treatment of extraskeletal myxoid chondrosarcoma may include the following:
Primitive neuroectodermal tumor (PNET)/extraskeletal Ewing tumor
See the PDQ summary on Ewing Sarcoma Treatment.
Treatment of synovial sarcoma may include the following:
Blood Vessel Tumors
Treatment of angiosarcoma may include the following:
Treatment of epithelioid hemangioendothelioma in the liver in infants aged younger than 1 year may include the following:
Treatment of children with epithelioid hemangioendothelioma that has spread may include:
Treatment of hemangiopericytoma (infantile) may include the following:
Metastatic Childhood Soft Tissue Sarcoma
Treatment of newly diagnosed metastatic childhood soft tissue sarcoma may include the following:
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with nonmetastatic childhood soft tissue sarcoma and metastatic childhood soft tissue sarcoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI Web site.
Recurrent and Progressive Childhood Soft Tissue Sarcoma
Treatment of recurrent or progressive childhood soft tissue sarcoma may include the following:
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with recurrent childhood soft tissue sarcoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI Web site.
For more information from the National Cancer Institute about childhood soft tissue sarcoma, see the following:
For more childhood cancer information and other general cancer resources, see the following:
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.
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National Cancer Institute: PDQ® Childhood Soft Tissue Sarcoma Treatment. Bethesda, MD: National Cancer Institute. Date last modified <MM/DD/YYYY>. Available at: http://cancer.gov/cancertopics/pdq/treatment/child-soft-tissue-sarcoma/Patient. Accessed <MM/DD/YYYY>.
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Last Revised: 2014-04-22
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