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Childhood Soft Tissue Sarcoma Treatment (PDQ)

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General Information About Childhood Soft Tissue Sarcoma
Stages of Childhood Soft Tissue Sarcoma
Recurrent and Progressive Childhood Soft Tissue Sarcoma
Treatment Option Overview
Treatment Options for Childhood Soft Tissue Sarcoma
To Learn More About Childhood Soft Tissue Sarcoma
Changes to This Summary (10/07/2014)
About This PDQ Summary
Get More Information From NCI

General Information About Childhood Soft Tissue Sarcoma

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:

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 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 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:

Stages of Childhood Soft Tissue Sarcoma

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 procedure may be used in the staging process:

The results of tests and procedures and the initial surgery are used 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:

Tumor size compared to everyday objects; shows various measurements of a tumor compared to a pea, peanut, walnut, and limePea, peanut, walnut, and lime show tumor sizes.

Stage I

Stage I is divided into stages IA and IB:

Stage II

Stage II is divided into stage IIA and stage IIB:

Stage III

In stage III, the tumor is either:

Stage IV

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 and Progressive Childhood Soft Tissue Sarcoma

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.

Treatment Option Overview

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

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

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

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 Soft Tissue Sarcoma for more information.

Watchful waiting

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

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

Targeted therapy is a type of treatment that uses drugs or other substances to attack cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation do.

Tyrosine kinase inhibitors (TKIs) are a type of targeted therapy that blocks signals needed for tumors to grow:

Other types of targeted therapy are being studied in clinical trials, including the following:

See Drugs Approved for Soft Tissue Sarcoma for more information.

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.

Treatment Options for Childhood Soft Tissue Sarcoma

Newly Diagnosed Childhood Soft Tissue Sarcoma

Fat Tissue Tumors

Liposarcoma

Treatment of liposarcoma may include the following:

Bone and Cartilage Tumors

Extraskeletal chondrosarcoma

Treatment of extraskeletal chondrosarcoma may include the following:

Extraskeletal osteosarcoma

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

Desmoid tumor

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:

Fibrosarcoma

Infantile fibrosarcoma

Treatment of infantile fibrosarcoma (also called congenital fibrosarcoma) may include the following:

Adult-type fibrosarcoma

Treatment of adult-type fibrosarcoma may include the following:

Dermatofibrosarcoma protuberans

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.

Myxofibrosarcoma, low-grade

Treatment of myxofibrosarcoma, low-grade may include the following:

Sclerosing epithelioid fibrosarcoma

Treatment of sclerosing epithelioid fibrosarcoma may include the following:

Skeletal Muscle Tumors

Rhabdomyosarcoma

See the PDQ summary on Childhood Rhabdomyosarcoma Treatment.

Smooth Muscle Tumors

Leiomyosarcoma

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:

Epithelioid sarcoma

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.

Synovial sarcoma

Treatment of synovial sarcoma may include the following:

Blood Vessel Tumors

Angiosarcoma (deep)

Treatment of angiosarcoma may include the following:

Epithelioid hemangioendothelioma

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:

Hemangiopericytoma (infantile)

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.

To Learn More About Childhood Soft Tissue Sarcoma

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:

Changes to This Summary (10/07/2014)

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.

About This PDQ Summary

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 ("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.

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 are listed in PDQ and can be found online at NCI's Web site. Many cancer doctors who take part in clinical trials are also listed in PDQ. For more information, call the Cancer Information Service 1-800-4-CANCER (1-800-422-6237).

Permission to Use This Summary

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The best way to cite this PDQ summary is:

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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Physicians version: CDR0000062934
Date first published: 2007-08-03 Date last modified: 2014-10-07

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