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This PDQ cancer information summary has current information about the treatment of childhood soft tissue sarcoma. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care.
Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Date Last Modified") is the date of the most recent change. The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Pediatric Treatment Editorial Board.
CONTENTS
- General Information About Childhood Soft Tissue Sarcoma
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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 tissue include the following:
Soft tissue sarcoma may be found anywhere in the body. In children, the tumors form most often in the arms, legs, chest, or abdomen.
Soft tissue sarcoma forms in soft tissues of the body, including muscle, tendons, fat, blood vessels, lymph vessels, nerves, and tissue around joints. 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 Soft Tissue Sarcoma for 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, the chest, or the abdomen. There may be no other signs or symptoms at first. As the sarcoma gets bigger and presses on nearby organs, nerves, muscles, or blood vessels, it may cause signs or symptoms, such as pain or weakness.
Other conditions may cause the same signs and symptoms. Check with your child’s doctor if your child has any of these problems.
Diagnostic tests are used to diagnose childhood soft tissue sarcoma.
The following tests and procedures may be used:
If tests show there may be a soft tissue sarcoma, a biopsy is done.
The type of biopsy depends, in part, on the size of the mass and whether it is close to the surface of the skin or deeper in the tissue. One of the following types of biopsies is usually used:
If possible, the surgeon who will remove any tumor that is found should be involved in planning the biopsy. The placement of needles or incisions for the biopsy can affect whether the whole tumor can be removed during later surgery.
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 cancer cells. 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, the tissue sample should be 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:
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
Liposarcoma. This is a cancer of the fat cells. Liposarcoma usually forms in the fat layer just under the skin. In children and adolescents, liposarcoma is often low grade (likely to grow and spread slowly). There are several different types of liposarcoma, including:
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:
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
Nerve sheath tumors
The nerve sheath is made up of protective layers of myelin that cover nerve cells that are not part of the brain or spinal cord. Nerve sheath tumors include the following types:
Pericytic (Perivascular) Tumors
Pericytic tumors form in cells that wrap around blood vessels. Pericytic tumors include the following types:
Tumors of unknown cell origin
Tumors of unknown cell origin (the type of cell the tumor first formed in 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:
Certain factors affect prognosis (chance of recovery) and treatment options.
The prognosis and treatment options depend on the following:
- Stages of Childhood Soft Tissue Sarcoma
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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.
To plan treatment, it is important to know the type of soft tissue sarcoma, whether the tumor can be removed by surgery, and whether cancer has spread to other parts of the body.
The following procedures may be used to find out if cancer has spread:
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.
Sometimes childhood soft tissue sarcoma continues to grow or comes back after treatment.
Progressive childhood soft tissue sarcoma is cancer that continues to grow, spread, or get worse. Progressive disease may be a sign that the cancer has become refractory to treatment.
Recurrent childhood soft tissue sarcoma is cancer that has recurred (come back) after treatment. The cancer may come back in the same place or in other parts of the body.
- Treatment Option Overview
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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:
Seven types of standard treatment are used:
Surgery
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. This is called neoadjuvant (preoperative) therapy.
The following types of surgery may be used:
A second surgery may be needed to:
If cancer is in the liver, a hepatectomy and liver transplant may be done (the liver is removed and replaced with a healthy one from a donor).
After the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy 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
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. There are two types of radiation therapy:
Whether the radiation therapy is given before or after surgery to remove the cancer depends on the type and stage of the cancer being treated, if any cancer cells remain after surgery, and the expected side effects of treatment. External and internal radiation therapy are used to treat childhood soft tissue sarcoma.
Chemotherapy
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.
Hyperthermic intraperitoneal chemotherapy (HIPEC) is a type of treatment used during surgery that is being studied for desmoplastic small round cell tumor. After the surgeon has removed as much tumor tissue as possible, warmed chemotherapy is sent directly into the peritoneal cavity.
The way the chemotherapy is given depends on the type of soft tissue sarcoma being treated. Most types of soft tissue sarcoma do not respond to treatment with chemotherapy.
See Drugs Approved for Soft Tissue Sarcoma for more information.
Observation
Observation is closely monitoring a patient’s condition without giving any treatment until signs or symptoms appear or change. Observation may be done when:
Observation may be used to treat desmoid-type fibromatosis, infantile fibrosarcoma, PEComa, or epithelioid hemangioendothelioma.
Targeted therapy
Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do.
New types of tyrosine kinase inhibitors are being studied such as:
Other types of targeted therapy are being studied in clinical trials, including the following:
See Drugs Approved for Soft Tissue Sarcoma for more information.
Immunotherapy
Immunotherapy is a treatment that uses the patient’s immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body’s natural defenses against cancer. This cancer treatment is a type of biologic therapy.
Interferon and immune checkpoint inhibitor therapy are types of immunotherapy.
Other Drug Therapy
Steroid therapy has antitumor effects in inflammatory myofibroblastic tumors.
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), such as tamoxifen, may be used to treat desmoid-type fibromatosis. Prasterone is being studied for the treatment of synovial sarcoma.
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 desmoid-type fibromatosis, an NSAID called sulindac may be used to help block the growth of cancer cells.
New types of treatment are being tested in clinical trials.
This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website.
Gene therapy
Gene therapy is being studied for childhood synovial sarcoma that has recurred, spread, or cannot be removed by surgery. Some of the patient's T cells (a type of white blood cell) are removed and the genes in the cells are changed in a laboratory (genetically engineered) so that they will attack specific cancer cells. They are then given back to the patient by infusion.
Treatment for childhood soft tissue sarcoma may cause side effects.
For information about side effects that begin during treatment for cancer, see our Side Effects page.
Side effects from cancer treatment that begin 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.)
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. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.
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.
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.
- Treatment of Childhood Soft Tissue Sarcoma
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For information about the treatments listed below, see the Treatment Option Overview section.
Fat Tissue Tumors
Liposarcoma
Treatment of newly diagnosed liposarcoma may include the following:
Bone and Cartilage Tumors
Extraskeletal mesenchymal chondrosarcoma
Treatment of newly diagnosed extraskeletal mesenchymal chondrosarcoma may include the following:
Extraskeletal osteosarcoma
Treatment of newly diagnosed extraskeletal osteosarcoma may include the following:
See the PDQ summary on Osteosarcoma and Malignant Fibrous Histiocytoma of Bone Treatment for more information about treatment of osteosarcoma.
Fibrous (Connective) Tissue Tumors
Desmoid-type fibromatosis
Treatment of newly diagnosed desmoid-type fibromatosis may include the following:
Dermatofibrosarcoma protuberans
Treatment of newly diagnosed dermatofibrosarcoma protuberans may include the following:
Inflammatory myofibroblastic tumor
Treatment of newly diagnosed inflammatory myofibroblastic tumor may include the following:
Fibrosarcoma
Infantile fibrosarcoma
Treatment of newly diagnosed infantile fibrosarcoma may include the following:
Adult fibrosarcoma
Treatment of adult newly diagnosed fibrosarcoma may include the following:
Myxofibrosarcoma
Treatment of newly diagnosed myxofibrosarcoma may include the following:
Low-grade fibromyxoid sarcoma
Treatment of newly diagnosed low-grade fibromyxoid sarcoma may include the following:
Sclerosing epithelioid fibrosarcoma
Treatment of newly diagnosed sclerosing epithelioid fibrosarcoma may include the following:
Skeletal Muscle Tumors
Rhabdomyosarcoma
See the PDQ summary on Childhood Rhabdomyosarcoma Treatment.
Smooth Muscle Tumors
Leiomyosarcoma
Treatment of newly diagnosed leiomyosarcoma may include the following:
So-called Fibrohistiocytic Tumors
Plexiform fibrohistiocytic tumor
Treatment of newly diagnosed plexiform fibrohistiocytic tumor may include the following:
Nerve Sheath Tumors
Malignant peripheral nerve sheath tumor
Treatment of newly diagnosed 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.
Malignant triton tumor
Newly diagnosed malignant triton tumors may be treated the same as rhabdomyosarcomas and include surgery, chemotherapy, or radiation therapy. It is not clear whether giving radiation therapy or chemotherapy improve the tumor's response to treatment.
Ectomesenchymoma
Treatment of newly diagnosed ectomesenchymoma may include the following:
Pericytic (Perivascular) Tumors
Infantile hemangiopericytoma
Treatment of newly diagnosed infantile hemangiopericytoma may include the following:
Infantile myofibromatosis
Treatment of newly diagnosed infantile myofibromatosis may include the following:
Tumors of Unknown Cell Origin (the place where the tumor first formed is not known)
Synovial sarcoma
Treatment of newly diagnosed synovial sarcoma may include the following:
Epithelioid sarcoma
Treatment of newly diagnosed epithelioid sarcoma may include the following:
Alveolar soft part sarcoma
Treatment of newly diagnosed alveolar soft part sarcoma may include the following:
Clear cell sarcoma of soft tissue
Treatment of newly diagnosed clear cell sarcoma of soft tissue may include the following:
Extraskeletal myxoid chondrosarcoma
Treatment of newly diagnosed extraskeletal myxoid chondrosarcoma may include the following:
Extraskeletal Ewing sarcoma
See the PDQ summary on Ewing Sarcoma Treatment.
Desmoplastic small round cell tumor
There is no standard treatment for newly diagnosed desmoplastic small round cell tumor. Treatment may include the following:
Extra-renal (extracranial) rhabdoid tumor
Treatment of newly diagnosed extra-renal (extracranial) rhabdoid tumor may include the following:
Perivascular epithelioid cell tumors (PEComas)
Treatment of newly diagnosed perivascular epithelioid cell tumors may include the following:
Undifferentiated/Unclassified Sarcoma
Undifferentiated pleomorphic sarcoma/malignant fibrous histiocytoma (high-grade)
There is no standard treatment for these tumors.
See the PDQ summary on Osteosarcoma Treatment for information about the treatment of malignant fibrous histiocytoma of bone.
Blood Vessel Tumors
Epithelioid hemangioendothelioma
Treatment of newly diagnosed epithelioid hemangioendothelioma may include the following:
Angiosarcoma of soft tissue
Treatment of newly diagnosed angiosarcoma may include the following:
Metastatic Childhood Soft Tissue Sarcoma
Treatment of childhood soft tissue sarcoma that has spread to other parts of the body at diagnosis may include the following:
For treatment of specific tumor types, see the Treatment Options for Childhood Soft Tissue Sarcoma section.
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.
- Treatment of Progressive or Recurrent Childhood Soft Tissue Sarcoma
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For information about the treatments listed below, see the Treatment Option Overview section.
Treatment of progressive or recurrent childhood soft tissue sarcoma may include the following:
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.
- To Learn More About Childhood Soft Tissue Sarcoma
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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:
- About This PDQ Summary
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About PDQ
Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. 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 come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish.
PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH.
Purpose of This Summary
This PDQ cancer information summary has current information about the treatment of childhood soft tissue sarcoma. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care.
Reviewers and Updates
Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Pediatric Treatment Editorial Board.
Clinical Trial Information
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 the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
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The best way to cite this PDQ summary is:
PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Soft Tissue Sarcoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/soft-tissue-sarcoma/patient/child-soft-tissue-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389342]
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