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:

  • Muscles
  • Tendons (bands of tissue that connect muscles to bones).
  • Synovial tissues (tissues around joints).
  • Fat
  • Blood vessels.
  • Lymph vessels.
  • Nerves

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.

Rhabdomyosarcoma is the most common type of childhood soft tissue sarcoma. It begins in muscles that surround bone. Rhabdomyosarcoma is not discussed in this document. This document is about the other types of soft tissue sarcoma:

Fibrous (connective) tissue tumors

  • Fibromatoses (desmoid tumor).
  • Dermatofibrosarcoma.
  • Fibrosarcoma.

Fibrohistiocytic tumors

  • Malignant fibrous histiocytoma (also called undifferentiated pleomorphic sarcoma, or spindle cell sarcoma).

Fat tissue tumors

  • Liposarcoma.

Smooth muscle tumors

  • Leiomyosarcoma.

Blood and lymph vessel tumors

  • Angiosarcoma.
  • Lymphangiosarcoma.
  • Hemangiopericytoma.
  • Hemangioendothelioma.

Peripheral nervous system tumors

  • Malignant schwannoma (malignant peripheral nerve sheath tumor).

Bone and cartilage tumors

  • Extraosseous osteosarcoma.
  • Extraosseous myxoid chondrosarcoma.
  • Extraosseus mesenchymal chondrosarcoma.

Tumors with more than one type of tissue

  • Malignant mesenchymoma.
  • Malignant Triton tumor.
  • Malignant ectomesenchymoma.

Tumors of unknown origin (the place where the tumor first formed is not known)

  • Alveolar soft part sarcoma.
  • Epithelioid sarcoma.
  • Clear cell sarcoma (malignant melanoma of soft parts).
  • Synovial sarcoma.
  • Desmoplastic small round cell tumor.

Besides rhabdomyosarcomas, the most common soft tissue sarcomas in children are in joint tissue, connective tissue, and nerve tissue.

Soft tissue sarcoma occurs in children and adults. Soft tissue sarcoma in children may respond differently to treatment, and may have a better outcome than soft tissue sarcoma in adults.

Having certain diseases and inherited disorders can increase the risk of developing 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. People who think they may be at risk should discuss this with their doctor. Risk factors for childhood soft tissue sarcoma include having the following inherited disorders:

  • Li-Fraumeni syndrome.
  • Neurofibromatosis type 1 (NF1).

Other risk factors include the following:

  • Having AIDS (acquired immune deficiency syndrome) and Epstein-Barr virus infection.
  • Having retinoblastoma in both eyes.
  • Past treatment with radiation therapy.
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 larger and presses on nearby organs, nerves, muscles, or blood vessels, symptoms may occur, including pain or weakness.

Other conditions may cause the same symptoms that soft tissue sarcomas do. A doctor should be consulted if any of these problems occur.

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.
  • 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 check the lump or painful area.
  • MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI).

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. A pathologist views the tissue or fluid under a microscope to look for cancer cells.
  • Core biopsy: The removal of tissue using a wide needle. A pathologist views the tissue under a microscope to look for cancer cells.
  • Incisional biopsy: The removal of part of a lump or a sample of tissue. A pathologist views the tissue under a microscope to look for cancer cells.
  • 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.

In order to plan the best treatment, a large sample of tissue may be removed during the biopsy to find out the type of soft tissue sarcoma and do 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 tumors usually grow and spread more quickly than low-grade tumors. Because soft tissue sarcoma can be hard to diagnose, patients should ask to have biopsy samples 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 study: A laboratory test in which dyes or enzymes are added to a blood or bone marrow sample to test for certain antigens (proteins that stimulate the body's immune response).
  • Immunocytochemistry study: A laboratory test that uses different substances to stain (color) cells in a sample of tissue. This is used to tell the difference between the 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 or whether the tumor has spread to other places in the body).
  • The location, grade, and size of the tumor and how deep under the skin the tumor is.
  • Whether or not the patient also has a condition called neurofibromatosis type 1 (NF1).
  • 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 amount of tumor remaining after surgery to remove the tumor and/or the grade and size of the tumor and whether it 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 may be used to stage childhood soft tissue sarcoma. This is the removal of the sentinel lymph node 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 radioactive substance and/or blue dye is injected near the tumor. The 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.

CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, 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.

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 determine the stage of the soft tissue sarcoma.

h6 There are three ways that cancer spreads in the body.

The three ways that cancer spreads in the body are:

  • Through tissue. Cancer invades the surrounding normal tissue.
  • Through the lymph system. Cancer invades the lymph system and travels through the lymph vessels to other places in the body.
  • Through the blood. Cancer invades the veins and capillaries and travels through the blood to other places in the body.

When cancer cells break away from the primary (original) tumor and travel through the lymph or blood to other places in the body, another (secondary) tumor may form. This process is called metastasis. The secondary (metastatic) tumor is the same type of cancer as the primary tumor. For example, if breast cancer spreads to the bones, the cancer cells in the bones are actually breast cancer cells. The disease is metastatic breast cancer, not bone 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, there are remaining cancer cells that can be seen only with a microscope.
  • Group III: After surgery, there is tumor remaining that can be seen with the eye.
Metastatic childhood soft tissue sarcoma
  • Group IV: The cancer has spread from where it started to other parts of the body (metastasis).

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.

Sometimes the stages used for adult soft tissue sarcoma are used for childhood soft tissue sarcoma:

Stage I
In stage I, the tumor is any size, low-grade (likely to grow and spread slowly), and may be either superficial (close to the skin's surface) or deep.

Stage II
In stage II, the tumor is high-grade (likely to grow and spread quickly) and either:

  • 5 centimeters or smaller and either superficial (close to the skin's surface) or deep; or
  • larger than 5 centimeters and superficial.

Stage III
In stage III, the tumor is high-grade, larger than 5 centimeters, and deep.

Stage IV
In stage IV, the tumor is any size, any grade, and has spread to nearby lymph nodes and/or to other parts of the body.

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:

  • Radiation oncologist.
  • Pediatric hematologist.
  • Pediatric nurse specialist.
  • Rehabilitation specialist.
  • Psychologist.
  • Social worker.
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.

Seven types of standard treatment are used:

Surgery
Surgery to completely remove the soft tissue sarcoma is done whenever 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 part or all of a limb or appendage, such as the arm or hand.
  • Limb-sparing surgery: Removal of the tumor in an arm or leg without amputation, so the use and appearance of the limb is saved. Radiation therapy or chemotherapy may be given first to shrink the tumor. The tumor is then removed in a wide local excision. Tissue and bone that are removed may be replaced with a graft using tissue and bone taken from another part of the patient's body, or with an implant such as artificial bone.
  • Lymphadenectomy: Removal of the lymph nodes that contain cancer.
  • Thoracotomy: An operation to open the chest. This may be done to remove cancer that has spread to the lungs.

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 them.

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. There are two types of radiation therapy. External radiation therapy uses a machine outside the body to send radiation toward the cancer. 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 spinal column, 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.

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.

Watchful waiting

Watchful waiting is closely monitoring a patient’s condition without giving any treatment until symptoms appear or change. Watchful waiting may be done when complete removal of the tumor is not possible, no other treatments are available, and the tumor does not place any vital organs in danger.

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.

Liver transplant

The liver is removed and replaced with a healthy one from a donor.

New types of treatment are being tested in clinical trials.

This 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 Web site.

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 (Gleevec) 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.

Other targeted therapies being studied in clinical trials include angiogenesis inhibitors. In cancer treatment, angiogenesis inhibitors prevent the growth of new blood vessels needed for tumors to grow.

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.

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 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

For some types or stages of cancer, there may not be any trials listed. Check with your doctor for clinical trials that are not listed here but may be right for you.

Nonmetastatic Childhood Soft Tissue Sarcoma

For treatment of fibrosarcoma or hemangiopericytoma

If an infant or young child has fibrosarcoma or hemangiopericytoma, treatment will be surgery whenever possible. Other treatments may include chemotherapy to reduce the size of the tumor, followed by surgery.

If an older child or adolescent has fibrosarcoma or hemangiopericytoma, treatment will be surgery whenever possible. Other treatments may include the following:

  • Chemotherapy followed by radiation therapy, surgery, or both.
  • Radiation therapy or chemotherapy after surgery.
  • A clinical trial of internal radiation therapy.
  • A clinical trial of radiation therapy given during surgery.

For treatment of desmoid tumor

Treatment of desmoid tumor will be surgery whenever possible. Other treatments may include watchful waiting, chemotherapy, or surgery followed by internal radiation therapy.

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

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

For treatment of alveolar soft part sarcoma

Treatment of alveolar soft part sarcoma will be surgery whenever possible. Other treatments may include the following:

  • Radiation therapy.
  • A clinical trial of surgery followed by watchful waiting, chemotherapy and/or radiation therapy, based on the grade of the tumor and the amount of tumor that is removed by surgery. Sometimes chemotherapy may be given before surgery.

For treatment of clear cell sarcoma of soft parts

Treatment of clear cell sarcoma of soft parts will be surgery whenever possible. Other treatments include radiation therapy after surgery.

For treatment of desmoplastic small round cell tumor

Treatment of desmoplastic small round cell tumor will be surgery whenever possible. Other treatments may include chemotherapy and radiation therapy after surgery.

For treatment of extraosseous osteosarcoma

See the NIH's PDQ summary on Osteosarcoma and Malignant Fibrous Histiocytoma of Bone Treatment for more information on the treatment of extraosseous osteosarcoma.

For treatment of hemangioendothelioma

Treatment of hemangioendothelioma in children younger than one year may include the following:

  • Watchful waiting as some tumors will disappear without treatment.
  • Treatment for blood clotting disorders.
  • Surgery to remove the tumor if there are symptoms.
  • Liver transplant (if the tumor is in the liver).

Treatment of hemangioendothelioma in children aged one year and older may include the following:

  • Surgery to remove the tumor.
  • Treatment for blood clotting disorders.
  • Liver transplant (if the tumor is in the liver).

For treatment of malignant peripheral nerve sheath tumor

Treatment of malignant peripheral nerve sheath tumor will be surgery whenever possible. Other treatments may include radiation therapy after surgery.

For treatment of synovial sarcoma

Treatment of synovial sarcoma may include the following:

  • Surgery with or without chemotherapy.
  • Chemotherapy.

For treatment of angiosarcoma and lymphangiosarcoma

Treatment of angiosarcoma and lymphangiosarcoma may include the following:

  • Surgery to completely remove the tumor.
  • Radiation therapy or chemotherapy after surgery.

For treatment of aggressive fibromatosis, dermatofibrosarcoma, and angiomatoid malignant fibrous histiocytoma

Treatment of these tumor types will be surgery whenever possible. Other treatments may include the following:

  • Targeted drug therapy with imatinib (Gleevec).
  • Radiation therapy after surgery.

For treatment of epithelioid sarcoma, leiomyosarcoma, liposarcoma, and mesenchymal chondrosarcoma

Treatment of these tumor types will be surgery whenever possible. Other treatments may include the following:

  • Chemotherapy followed by radiation therapy, surgery, or both.
  • Radiation therapy or chemotherapy after surgery.
  • A clinical trial of internal radiation therapy.
  • A clinical trial of radiation therapy given during surgery.
Metastatic Childhood Soft Tissue Sarcoma

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

  • Combination chemotherapy, radiation therapy, and surgery (thoracotomy) to remove cancer that has spread to the lungs.
  • A clinical trial of combination chemotherapy and granulocyte colony-stimulating factor (substance that helps the body increase the number of white blood cells).
  • A clinical trial of a targeted therapy.
Recurrent and Progressive Childhood Soft Tissue Sarcoma

Treatment of recurrent or progressive childhood soft tissue sarcoma may include the following:

  • Watchful waiting for some desmoid tumors.
  • Surgery followed by radiation therapy, if not already given.
  • Surgery to remove the arm or leg with cancer, if radiation therapy was already given.
  • Surgery to remove cancer that has spread to the lungs.
  • A clinical trial of new combinations of chemotherapy.
  • A clinical trial of targeted drug therapy with imatinib (Gleevec).

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with 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. General information about clinical trials is available from the NCI Web site.

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

NCI Public Inquiries Office
Suite 3036A
6116 Executive Boulevard, MSC8322
Bethesda, MD 20892-8322

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 9:00 a.m. to 4:30 p.m. Deaf and hard-of-hearing callers with TTY equipment may call 1-800-332-8615. Information about ongoing clinical trials is available from the NCI Web site.

Source: National Institutes of Health, National Cancer Institute

This information is provided by the Cleveland Clinic and is not intended to replace the medical advice of your doctor or health care provider. Please consult your health care provider for advice about a specific medical condition. For additional written health information, please contact the Health Information Center at the Cleveland Clinic (216) 444-3771 or toll-free (800) 223-2273 extension 43771 or visit www.clevelandclinic.org/health/.

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