Nonmetastatic Childhood Soft Tissue Sarcoma
Metastatic Childhood Soft Tissue Sarcoma
Recurrent and Progressive Childhood Soft Tissue Sarcoma

A link to a list of current clinical trials is included for each treatment section. 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.

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.
• 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.

• If surgery to completely remove the tumor is not possible, treatment may include the following:
  • External radiation therapy to shrink the tumor before surgery.
  • Internal radiation therapy to kill any remaining tumor cells after surgery.
  • A nonsteroidal anti-inflammatory drug (NSAID).
  • Antiestrogen therapy.
  • Chemotherapy.
• Watchful waiting.
• A clinical trial of targeted drug therapy with imatinib (Gleevec).

For treatment of malignant peripheral nerve sheath tumor, liposarcoma, synovial sarcoma, extraosseous osteosarcoma, extraosseous chondrosarcoma, malignant fibrous histiocytoma, leiomyosarcoma, or epithelioid sarcoma

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

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

Synovial sarcoma may also be treated with surgery followed by chemotherapy. Malignant peripheral nerve sheath tumor may be treated with radiation therapy after surgery.

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 after surgery.
• A clinical trial of surgery with or without chemotherapy and/or radiation therapy, based on the grade of the tumor and the amount of tumor that is removed by 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 clear cell sarcoma of soft parts

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

For treatment of hemangioendothelioma

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).

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.

For treatment of angiosarcoma and lymphangiosarcoma

Treatment of angiosarcoma and lymphangiosarcoma may include the following:

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

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with nonmetastatic childhood soft tissue sarcoma ¹. 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 ².

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).

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with metastatic childhood soft tissue sarcoma ³. 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 ².

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

• 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 chemotherapy or targeted drug therapy with imatinib (Gleevec).
• A clinical trial of a new treatment.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with recurrent childhood soft tissue sarcoma ⁴. 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 ².

Glossary Terms

A type of cancer that begins in the cells that line blood vessels or lymph vessels. Cancer that begins in blood vessels is called hemangiosarcoma. Cancer that begins in lymph vessels is called lymphangiosarcoma.

A substance that prevents cells from making or using estrogen (a hormone that plays a role in the formation of female sex characteristics, the menstrual cycle, and pregnancy). Antiestrogens may stop some cancer cells from growing and are used to prevent and treat breast cancer. They are also being studied in the treatment of other types of cancer. An antiestrogen is a type of hormone antagonist.

A mass of blood that forms when blood platelets, proteins, and cells stick together. When a blood clot is attached to the wall of a blood vessel, it is called a thrombus. When it moves through the bloodstream and blocks the flow of blood in another part of the body, it is called an embolus.

A term for diseases in which abnormal cells divide without control. Cancer cells can invade nearby tissues and can spread to other parts of the body through the blood and lymph systems. There are several main types of cancer. Carcinoma is cancer that begins in the skin or in tissues that line or cover internal organs. Sarcoma is cancer that begins in bone, cartilage, fat, muscle, blood vessels, or other connective or supportive tissue. Leukemia is cancer that starts in blood-forming tissue such as the bone marrow, and causes large numbers of abnormal blood cells to be produced and enter the blood. Lymphoma and multiple myeloma are cancers that begin in the cells of the immune system. Central nervous system cancers are cancers that begin in the tissues of the brain and spinal cord.

The individual unit that makes up the tissues of the body. All living things are made up of one or more cells.

Treatment with drugs that kill cancer cells.

A type of cell that looks clear inside when viewed under a microscope.

A type of research study that tests how well new medical approaches work in people. These studies test new methods of screening, prevention, diagnosis, or treatment of a disease. Also called clinical study.

Treatment using more than one anticancer drug.

A tumor of the tissue that surrounds muscles, usually in the abdomen. A desmoid tumor rarely metastasizes (spreads to other parts of the body). It may be called aggressive fibromatosis when the tumor is outside of the abdomen.

A rare, aggressive cancer that usually affects young males and usually is located in the abdomen.

In medicine, a disturbance of normal functioning of the mind or body. Disorders may be caused by genetic factors, disease, or trauma.

A type of radiation therapy that uses a machine to aim high-energy rays at the cancer from outside of the body. Also called external-beam radiation therapy.

A type of soft tissue sarcoma that begins in fibrous tissue, which holds bones, muscles, and other organs in place.

The grade of a tumor depends on how abnormal the cancer cells look under a microscope and how quickly the tumor is likely to grow and spread. Grading systems are different for each type of cancer.

A colony-stimulating factor that stimulates the production of neutrophils (a type of white blood cell). It is a cytokine that is a type of hematopoietic (blood-forming) agent. Also called filgrastim and G-CSF.

A type of cancer involving blood vessels and soft tissue.

A drug used to treat different types of leukemia and other cancers of the blood, gastrointestinal stromal tumors, skin tumors called dermatofibrosarcoma protuberans, and a rare condition called systemic mastocytosis. It is also being studied in the treatment of other types of cancer. Imatinib mesylate blocks the protein made by the bcr/abl oncogene. It is a type of tyrosine kinase inhibitor. Also called Gleevec and STI571.

A type of radiation therapy in which radioactive material sealed in needles, seeds, wires, or catheters is placed directly into or near a tumor. Also called brachytherapy, implant radiation therapy, and radiation brachytherapy.

A large organ located in the upper abdomen. The liver cleanses the blood and aids in digestion by secreting bile.

One of a pair of organs in the chest that supplies the body with oxygen, and removes carbon dioxide from the body.

A type of cancer that begins in the cells that line lymph vessels.

A type of soft tissue sarcoma that develops in cells that form a protective sheath (covering) around peripheral nerves, which are nerves that are outside of the central nervous system (brain and spinal cord). Also called MPNST.

Having to do with metastasis, which is the spread of cancer from one part of the body to another.

A drug that decreases fever, swelling, pain, and redness. Also called NSAID.

Cancer that is growing, spreading, or getting worse.

The use of high-energy radiation from x-rays, gamma rays, neutrons, protons, and other sources to kill cancer cells and shrink tumors. Radiation may come from a machine outside the body (external-beam radiation therapy), or it may come from radioactive material placed in the body near cancer cells (internal radiation therapy). Systemic radiation therapy uses a radioactive substance, such as a radiolabeled monoclonal antibody, that travels in the blood to tissues throughout the body. Also called irradiation and radiotherapy.

Cancer that has recurred (come back), usually after a period of time during which the cancer could not be detected. The cancer may come back to the same place as the original (primary) tumor or to another place in the body. Also called recurrence.

A cancer of the bone, cartilage, fat, muscle, blood vessels, or other connective or supportive tissue.

A cancer that begins in the muscle, fat, fibrous tissue, blood vessels, or other supporting tissue of the body.

The extent of a cancer in the body. Staging is usually based on the size of the tumor, whether lymph nodes contain cancer, and whether the cancer has spread from the original site to other parts of the body.

A procedure to remove or repair a part of the body or to find out whether disease is present. An operation.

An indication that a person has a condition or disease. Some examples of symptoms are headache, fever, fatigue, nausea, vomiting, and pain.

A malignant tumor that develops in the synovial membrane of the joints.

A type of treatment that uses drugs or other substances, such as monoclonal antibodies, to identify and attack specific cancer cells without harming normal cells. Targeted therapy may be less harmful to normal cells than other types of cancer treatments

Treatment.

An operation to open the chest.

A surgical procedure in which tissue or an organ is transferred from one area of a person’s body to another area, or from one person (the donor) to another person (the recipient).

An abnormal mass of tissue that results when cells divide more than they should or do not die when they should. Tumors may be benign (not cancerous), or malignant (cancerous). Also called neoplasm.

Closely monitoring a patient's condition but withholding treatment until symptoms appear or change. Also called active surveillance, expectant management, and observation.

Refers to a blood cell that does not contain hemoglobin. White blood cells include lymphocytes, neutrophils, eosinophils, macrophages, and mast cells. These cells are made by bone marrow and help the body fight infections and other diseases. Also called leukocyte and WBC.