Childhood Soft Tissue Sarcoma Treatment (PDQ®): Treatment - Patient Information [NCI]

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Childhood Soft Tissue Sarcoma Treatment

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:

  • Fat.
  • A mix of bone and cartilage tissue.
  • Fibrous tissue.
  • Muscles.
  • Nerves.
  • Tendons (bands of tissue that connect muscles to bones).
  • Synovial tissues (tissues around joints).
  • Blood vessels.
  • Lymph vessels.

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

  • Liposarcoma. This is a rare cancer of the fat cells. There are several different types of liposarcoma. Most liposarcomas in children and teenagers are low grade (likely to grow and spread slowly). The most common type is myxoid liposarcoma, which is usually low grade and responds well to treatment. Another type is pleomorphic liposarcoma, which is usually high grade (likely to grow and spread quickly) and is less likely to respond well to treatment.

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:

  • Extraskeletalchondrosarcoma. This type of bone and cartilage tumor includes mesenchymal chondrosarcoma, which grows quickly and often spreads to the lungs.
  • Extraskeletal osteosarcoma. This type of bone and cartilage tumor is very rare in children and teenagers. It is likely to come back after treatment and may spread to the lungs.

Fibrous (connective) tissue tumors

Fibrous (connective) tissue tumors include the following types:

  • Desmoid tumor (also called aggressive fibromatosis). This fibrous tissue tumor is low grade (likely to grow slowly). This tumor may come back in nearby tissues but usually does not spread to distant parts of the body. Rarely, the tumor may disappear without treatment.

    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.

  • Fibrosarcoma.

    There are two types of fibrosarcoma in children and teenagers:

    • Infantile fibrosarcoma (also called congenital fibrosarcoma). This type of fibrosarcoma is found in children aged 4 years and younger. It most often forms in infants and may be seen in a prenatal ultrasound exam. This tumor is often large and fast growing, but rarely spreads to distant parts of the body. The cells of this tumor usually have a certain genetic change called a translocation (part of one chromosome switches places with part of another chromosome). In order to diagnose infantile fibrosarcoma, the tumor cells are checked for this genetic change.
    • Adult-type fibrosarcoma. This is the same type of fibrosarcoma found in adults. The cells of this tumor do not have the genetic change found in infantile fibrosarcoma.
  • Dermatofibrosarcoma protuberans. This is a rare fibrous tissue tumor found in children and adults. The cells of this tumor have a certain genetic change called a translocation. In order to diagnose dermatofibrosarcoma protuberans, the tumor cells are checked for this genetic change.
  • Inflammatory myofibroblastic tumor. This is a fibrous tissue tumor that is likely to come back after treatment but rarely spreads to distant parts of the body. A certain genetic change has been found in about half of these tumors.
  • Low-grade fibromyxoid sarcoma. This tumor may come back many years after treatment and is likely to spread to the lungs and the lining of the wall of the chest cavity. Lifelong follow-up is needed.
  • Myxofibrosarcoma, low-grade. This is a rare fibrous tissue tumor that is found less often in children than in adults. It is likely to grow slowly.
  • Sclerosing epithelioid fibrosarcoma. This is a rare fibrous tissue tumor that usually grows slowly.

Skeletal muscle tumors

Skeletal muscle is attached to bones and helps the body move.

  • Rhabdomyosarcoma. Rhabdomyosarcoma is the most common childhood soft tissue sarcoma. See the PDQ summary on Childhood Rhabdomyosarcoma Treatment for more information.

Smooth muscle tumors

Smooth muscle lines the inside of blood vessels and hollow internal organs such as the stomach, intestines, bladder, and uterus.

  • Leiomyosarcoma. This smooth muscle tumor has been linked with Epstein-Barr virus in children who also have HIV /AIDS. Leiomyosarcoma may also form as a second cancer in survivors of inherited retinoblastoma, even many years after their treatment for retinoblastoma.

So-called fibrohistiocytic tumors

So-called fibrohistiocytic tumors include the following types:

  • Plexiform fibrohistiocytic tumor. This is a rare tumor that usually affects children and young adults. The tumor usually starts as a painless growth on or just under the skin on the arm, hand, or wrist. It may rarely spread to nearby lymph nodes or to the lungs.
  • Undifferentiated pleomorphic sarcoma. This type of tumor may form in parts of the body where patients have received radiation therapy in the past, or as a second cancer in children with retinoblastoma. The tumor is usually found on the arms or legs and may spread to other parts of the body.

Peripheral nervous system tumors

  • Malignant peripheral nerve sheath tumor. This is a tumor that forms in the protective covering of nerves that are not part of the brain or spinal cord. Some children who have a malignant peripheral nerve sheath tumor have a rare genetic condition called neurofibromatosis type 1 (NF1). This tumor may be low grade or high grade.

Tumors of unknown origin

Tumors of unknown origin (the place where the tumor first formed is not known) include the following types:

  • Alveolar soft part sarcoma. This is a rare tumor of the soft supporting tissue that connects and surrounds the organs and other tissues. It may grow slowly and has often spread to other parts of the body at the time of diagnosis. Alveolar soft part sarcoma may have a better prognosis in children than in adults, when the tumor is 5 centimeters or smaller, or when the tumor is completely removed by surgery.
  • Clear cell sarcoma of soft tissue. This is a soft tissue tumor that begins in a tendon (tough, fibrous, cord-like tissue that connects muscle to bone or to another part of the body). Clear cell sarcoma may spread to nearby lymph nodes.
  • Desmoplastic small round cell tumor. This tumor most often affects boys and may form and spread in the abdomen and pelvis or tissues around the testes. Desmoplastic small round cell tumor may also spread to the lungs and other parts of the body.
  • Epithelioid sarcoma. This is a rare sarcoma that usually starts deep in soft tissue as a slow growing, firm lump and may spread to the lymph nodes.
  • Perivascular epithelioid cell tumors (PEComas). Benign (not cancer) PEComas are commonly found in children with an inherited condition called tuberous sclerosis.
  • Extrarenal (extracranial) rhabdoid tumor. This is a rare, fast-growing tumor of soft tissues such as the liver and peritoneum. It usually occurs in young children, including newborns, but it can occur in older children and adults.
  • Extraskeletal myxoid chondrosarcoma. This is a rare soft tissue sarcoma that may be found in children and teenagers. This tumor may come back after treatment. Over time, it tends to spread to other parts of the body including the lymph nodes and the lungs.
  • Primitive neuroectodermal tumor (PNET)/extraskeletalEwing tumor. See the PDQ summary on Ewing Sarcoma Treatment for more information.
  • Synovial sarcoma. Synovial sarcoma is a common type of soft tissue sarcoma in children and teenagers. Synovial sarcoma usually forms in the tissues around the joints in the arms or legs, but may also form in the trunk, head, or neck. The cells of this tumor usually have a certain genetic change called a translocation (part of one chromosome switches places with part of another chromosome). Larger tumors have a greater risk of spreading to other parts of the body, including the lungs. Children younger than 10 years and those whose tumor is 5 centimeters or smaller have a better prognosis.

Blood vessel tumors

Blood vessel tumors include the following types:

  • Angiosarcoma (deep). Angiosarcomas may form inside blood vessels or lymph vessels. This type of tumor usually grows quickly.
  • Epithelioid hemangioendothelioma. In infants, these tumors often form in the liver but may form in other parts of the body such as the lung. They are usually benign (not cancer). Children with consumptive coagulopathy (also known as Kasabach-Merritt syndrome) may have an increased chance of epithelioid hemangioendothelioma. In children younger than 1 year, the tumor may go away without treatment. A small number of children may develop cancerous tumors over time. Infants with hemangiomas should be checked regularly with ultrasound exams and may be treated with a liver transplant if their tumors are benign. In some cases, the benign tumor may become malignant (cancer) and spread to the lungs, lymph nodes, bones, abdomen, or pelvis.
  • Hemangiopericytoma (infantile). This soft tissue tumor has many blood vessels. Children younger than 1 year have a better prognosis. In patients older than 1 year, this tumor is more likely to spread to distant tissues, including the lymph nodes and lungs.

See the following PDQ summaries for information about types of soft tissue sarcoma not included in this summary:

  • Childhood Rhabdomyosarcoma Treatment.
  • Ewing Sarcoma Treatment.
  • Unusual Cancers of Childhood (gastrointestinal stromal tumors).

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:

  • Li-Fraumeni syndrome.
  • Neurofibromatosis type 1 (NF1).
  • Familial adenomatous polyposis (FAP).
  • Werner syndrome.
  • Retinoblastoma gene changes.

Other risk factors include the following:

  • Past treatment with radiation therapy.
  • Having AIDS (acquired immune deficiency syndrome) and Epstein-Barr virus infection at the same time.

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:

  • Physical exam and history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient's health habits and past illnesses and treatments will also be taken.
  • CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the abdomen, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
  • MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas of the body, such as the chest, abdomen, arms, or legs. This procedure is also called nuclear magnetic resonance imaging (NMRI).
    Magnetic resonance imaging (MRI) of the abdomen; drawing shows the patient on a table that slides into the MRI machine, which takes pictures of the inside of the body. The pad on the patient's abdomen helps make the pictures clearer.
    Magnetic resonance imaging (MRI) of the abdomen. The patient lies on a table that slides into the MRI machine, which takes pictures of the inside of the body. The pad on the patient's abdomen helps make the pictures clearer.
  • Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. The picture can be printed to be looked at later.

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:

  • Fine-needle aspiration (FNA) biopsy: The removal of tissue or fluid using a thin needle.
  • Core needle biopsy: The removal of tissue using a wide needle. This procedure may be guided using ultrasound, CT scan, or MRI.
  • Incisional biopsy: The removal of part of a lump or a sample of tissue.
  • Excisional biopsy: The removal of an entire lump or area of tissue that doesn't look normal. A pathologist views the tissue under a microscope to look for cancer cells. An excisional biopsy may be used to completely remove smaller tumors that are near the surface of the skin. This type of biopsy is rarely used because cancer cells may remain after the biopsy. If cancer cells remain, the cancer may come back or it may spread to other parts of the body.

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:

  • Cytogenetic analysis: A laboratory test in which cells in a sample of tissue are viewed under a microscope to look for certain changes in the chromosomes.
  • Immunohistochemistry: A test that uses antibodies to check for certain antigens in a sample of blood or bone marrow. The antibody is usually linked to a radioactive substance or a dye that causes the tissue to light up under a microscope. This type of test may be used to tell the difference between different types of cancer.
  • Immunocytochemistry: A test that uses antibodies to check for certain antigens in a sample of cells. The antibody is usually linked to a radioactive substance or a dye that causes the cells to light up under a microscope. This type of test may be used to tell the difference between different types of soft tissue sarcoma.
  • Light and electron microscopy: A laboratory test in which cells in a sample of tissue are viewed under regular and high-powered microscopes to look for certain changes in the cells.

Certain factors affect prognosis (chance of recovery) and treatment options.

The prognosis (chance of recovery) and treatment options depend on the following:

  • The type of soft tissue sarcoma.
  • The stage of the cancer (the amount of tumor remaining after surgery to remove it and whether the tumor has spread to other places in the body).
  • The grade and size of the tumor, what part of the body it's in, and how deep under the skin it is.
  • The age of the patient.
  • Whether the cancer has just been diagnosed or has recurred (come back).

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:

  • Amount of tumor remaining after surgery to remove the tumor; and/or
  • Grade and size of the tumor; and
  • Whether the tumor has spread to the lymph nodes or other parts of the body.

It is important to know the stage in order to plan treatment.

The following tests and procedures may be used in the staging process:

  • Sentinel lymph node biopsy: A sentinel lymph node biopsy is done to check if cancer has spread to the lymph nodes. The sentinel lymph node is removed during surgery. The sentinel lymph node is the first lymph node to receive lymphatic drainage from a tumor. It is the first lymph node the cancer is likely to spread to from the tumor. A small amount of a radioactive substance and/or blue dye is injected near the tumor. The radioactive substance or dye flows through the lymph ducts to the lymph nodes. The first lymph node to receive the substance or dye is removed. A pathologist views the tissue under a microscope to look for cancer cells. If cancer cells are not found, it may not be necessary to remove more lymph nodes. This procedure is used for epithelioid and clear cell sarcoma.
  • CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the chest, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
  • X-rays: An x-ray is a type of energy beam that can go through the body onto film, making pictures of areas inside the body. A series of x-rays may be done to see if cancer has spread to the bone.

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:

  • Tissue. The cancer spreads from where it began by growing into nearby areas.
  • Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body.
  • Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body.

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.

  • Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body.
  • Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body.

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.

  • Group I: The tumor has been completely removed by surgery.
  • Group II: After surgery to remove the tumor, cancer cells remain that can only be seen with a microscope at the edge of the tissue removed and/or in nearby lymph nodes.
  • Group III: After surgery or biopsy, there is tumor remaining that has not spread but can be seen with the eye.

Metastatic childhood soft tissue sarcoma

  • Group IV: The cancer has metastasized (spread from where it started to other parts of the body).

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:

  • The size of the tumor.
  • Whether the tumor has spread to the lymph nodes.
  • Whether the tumor has spread to other parts of the body.

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

Stage I

Stage I is divided into stages IA and IB:

  • In stage IA, the tumor is low grade (likely to grow and spread slowly) and 5 centimeters or smaller.
  • In stage IB, the tumor is low grade (likely to grow and spread slowly) and larger than 5 centimeters.

Stage II

Stage II is divided into stage IIA and stage IIB:

  • In stage IIA, the tumor is mid grade (somewhat likely to grow and spread quickly) or high grade (likely to grow and spread quickly) and 5 centimeters or smaller.
  • In stage IIB, the tumor is mid grade (somewhat likely to grow and spread quickly) and larger than 5 centimeters.

Stage III

In stage III, the tumor is either:

  • high grade (likely to grow and spread quickly) and larger than 5 centimeters; or
  • any grade or size, and has spread to nearby lymph nodes.

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:

  • Pediatrician.
  • Radiation oncologist.
  • Pediatric hematologist.
  • Pediatric nurse specialist.
  • Rehabilitation specialist.
  • Psychologist.
  • Social worker.
  • Child-life specialist.

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:

  • Physical problems.
  • Changes in mood, feelings, thinking, learning, or memory.
  • Second cancers (new types of cancer).

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:

  • Wide local excision: Removal of the tumor along with some normal tissue around it.
  • Amputation: Surgery to remove all or part of the limb or appendage with cancer, such as the arm or hand.
  • Lymphadenectomy: Removal of the lymph nodes with cancer.

A second surgery may be needed to:

  • Remove any remaining cancer cells.
  • Check the area around where the tumor was removed for cancer cells and then remove more tissue if needed.

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:

  • External radiation therapy uses a machine outside the body to send radiation toward the cancer. Stereotactic radiation therapy is a type of external radiation therapy that aims radiation directly to a tumor, causing less damage to normal tissue around the tumor. The total dose of radiation is divided into several smaller doses given over several days. This procedure is also called stereotactic external-beam radiation therapy and stereotaxic radiation therapy.
  • Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer.

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 Adult and Childhood 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:

  • Complete removal of the tumor is not possible.
  • No other treatments are available.
  • The tumor does not place any vital organs in danger.

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 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.

Treatment Options for Childhood Soft Tissue Sarcoma

Newly Diagnosed Childhood Soft Tissue Sarcoma

Fat Tissue Tumors

Liposarcoma

Treatment of liposarcoma may include the following:

  • Surgery to completely remove the tumor. If the cancer is not completely removed, a second surgery may be done.
  • Chemotherapy to shrink the tumor, followed by surgery.

Bone and Cartilage Tumors

Extraskeletal chondrosarcoma

Treatment of extraskeletal chondrosarcoma may include the following:

  • Surgery to completely remove the tumor.
  • Surgery to remove as much of the tumor as possible, followed by radiation therapy.
  • Combination chemotherapy may be given after surgery to lessen the chance the tumor will spread to the lungs.

Extraskeletal osteosarcoma

Treatment of extraskeletal osteosarcoma may include the following:

  • Surgery to completely remove the tumor.

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:

  • Surgery to completely remove the tumor when possible. Surgery may be followed by radiation therapy if tumor remains.
  • Radiation therapy, if the tumor cannot be removed by surgery.
  • Watchful waiting.

If surgery to completely remove the tumor is not possible, treatment to shrink the tumor before surgery may include the following:

  • Chemotherapy.
  • Antiestrogen therapy.
  • A nonsteroidal anti-inflammatory drug (NSAID).
  • External radiation therapy.

Fibrosarcoma

Infantile fibrosarcoma

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

  • Surgery to remove the tumor when possible.
  • Chemotherapy to shrink the tumor, followed by surgery.

Adult-type fibrosarcoma

Treatment of adult-type fibrosarcoma may include the following:

  • Surgery to completely remove the tumor when possible.

Dermatofibrosarcoma protuberans

Treatment of dermatofibrosarcoma protuberans may include the following:

  • Surgery to completely remove the tumor when possible.
  • Radiation therapy if the tumor is not completely removed.
  • Targeted therapy (imatinib) if the tumor cannot be removed or has come back.

Inflammatory myofibroblastic tumor

Treatment of inflammatory myofibroblastic tumor may include the following:

  • Surgery to completely remove the tumor when possible.

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:

  • Surgery to completely remove the tumor.

Sclerosing epithelioid fibrosarcoma

Treatment of sclerosing epithelioid fibrosarcoma may include the following:

  • Surgery to completely remove the tumor.

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:

  • Surgery to completely remove the tumor.

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:

  • Surgery to completely remove the tumor when possible.

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:

  • Surgery to completely remove the tumor when possible.
  • Radiation therapy if the tumor cannot be completely removed by surgery.
  • A clinical trial of targeted therapy.

Clear cell sarcoma of soft tissue

Treatment of clear cell sarcoma of soft tissue may include the following:

  • Surgery to remove the tumor when possible. Radiation therapy may be given after surgery.

Desmoplastic small round cell tumor

There is no standard treatment for desmoplastic small round cell tumor. Treatment may include the following:

  • A combination of chemotherapy, surgery, and radiation therapy.

Epithelioid sarcoma

Treatment of epithelioid sarcoma may include the following:

  • Surgery to remove the tumor when possible.
  • Chemotherapy.
  • Radiation therapy.

Perivascular epithelioid cell tumors (PEComas)

Treatment of perivascular epithelioid cell tumors may include the following:

  • Surgery to remove the tumor.
  • Targeted therapy (mTOR inhibitor therapy).

Extrarenal (extracranial) rhabdoid tumor

Treatment of extrarenal (extracranial) rhabdoid tumor may include the following:

  • A combination of surgery to remove the tumor when possible, chemotherapy, and radiation therapy.

Extraskeletal myxoid chondrosarcoma

Treatment of extraskeletal myxoid chondrosarcoma may include the following:

  • Surgery to remove the tumor when possible.
  • A clinical trial of targeted therapy.

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:

  • Surgery with or without radiation therapy, followed by adjuvant chemotherapy.

Blood Vessel Tumors

Angiosarcoma (deep)

Treatment of angiosarcoma may include the following:

  • Surgery to completely remove the tumor.
  • Chemotherapy.
  • A clinical trial of an angiogenesis inhibitor.

Epithelioid hemangioendothelioma

Treatment of epithelioid hemangioendothelioma in the liver in infants aged younger than 1 year may include the following:

  • For infants who do not have signs or symptoms: Watchful waiting because some tumors will disappear without treatment.
  • For infants who have signs or symptoms: Surgery is usually done to remove the tumor.
  • Liver transplant.

Treatment of children with epithelioid hemangioendothelioma that has spread may include:

  • A clinical trial of a targeted therapy (mTor inhibitor).

Hemangiopericytoma (infantile)

Treatment of hemangiopericytoma (infantile) may include the following:

  • Chemotherapy.

Metastatic Childhood Soft Tissue Sarcoma

Treatment of newly diagnosed metastatic childhood soft tissue sarcoma may include the following:

  • Combination chemotherapy, radiation therapy, and surgery to remove the tumor. Surgery may also be done to remove tumors that have spread to other parts of the body such as the lungs.
  • Stereotactic radiation therapy for tumors that have spread to other parts of the body.
  • A clinical trial of a targeted therapy.

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:

  • Surgery followed by external or internal radiation therapy, if radiation therapy has not already been given.
  • Surgery to remove the arm or leg with cancer, if radiation therapy was already given.
  • Surgery to remove cancer that has come back where it first formed or has spread to the lungs.
  • Chemotherapy.
  • Targeted therapy.
  • A clinical trial of a new treatment.

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:

  • Soft Tissue Sarcoma Home Page
  • Computed Tomography (CT) Scans and Cancer
  • Drugs Approved for Adult and Childhood Soft Tissue Sarcoma
  • Targeted Cancer Therapies

For more childhood cancer information and other general cancer resources, see the following:

  • Childhood Cancers
  • CureSearch for Children's Cancer
  • Late Effects of Treatment for Childhood Cancer
  • Adolescents and Young Adults with Cancer
  • Young People with Cancer: A Handbook for Parents
  • Care for Children and Adolescents with Cancer
  • Cancer Staging
  • Coping with Cancer: Supportive and Palliative Care
  • Questions to Ask Your Doctor About Cancer
  • Cancer Library
  • Information for Survivors/Caregivers/Advocates

Changes to This Summary (04 / 22 / 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

PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as "NCI's PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary]."

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>.

Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 2,000 scientific images.

Disclaimer

The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Coping with Cancer: Financial, Insurance, and Legal Information page.

Contact Us

More information about contacting us or receiving help with the Cancer.gov Web site can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the Web site's Contact Form.

Get More Information From NCI

Call 1-800-4-CANCER

For more information, U.S. residents may call the National Cancer Institute's (NCI's) Cancer Information Service toll-free at 1-800-4-CANCER (1-800-422-6237) Monday through Friday from 8:00 a.m. to 8:00 p.m., Eastern Time. A trained Cancer Information Specialist is available to answer your questions.

Chat online

The NCI's LiveHelp® online chat service provides Internet users with the ability to chat online with an Information Specialist. The service is available from 8:00 a.m. to 11:00 p.m. Eastern time, Monday through Friday. Information Specialists can help Internet users find information on NCI Web sites and answer questions about cancer.

Write to us

For more information from the NCI, please write to this address:

NCI Public Inquiries Office
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Room 2E532 MSC 9760
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Search the NCI Web site

The NCI Web site provides online access to information on cancer, clinical trials, and other Web sites and organizations that offer support and resources for cancer patients and their families. For a quick search, use the search box in the upper right corner of each Web page. The results for a wide range of search terms will include a list of "Best Bets," editorially chosen Web pages that are most closely related to the search term entered.

There are also many other places to get materials and information about cancer treatment and services. Hospitals in your area may have information about local and regional agencies that have information on finances, getting to and from treatment, receiving care at home, and dealing with problems related to cancer treatment.

Find Publications

The NCI has booklets and other materials for patients, health professionals, and the public. These publications discuss types of cancer, methods of cancer treatment, coping with cancer, and clinical trials. Some publications provide information on tests for cancer, cancer causes and prevention, cancer statistics, and NCI research activities. NCI materials on these and other topics may be ordered online or printed directly from the NCI Publications Locator. These materials can also be ordered by telephone from the Cancer Information Service toll-free at 1-800-4-CANCER (1-800-422-6237).

Last Revised: 2014-04-22


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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