Treatment Options for Childhood Soft Tissue Sarcoma
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.
Nonmetastatic Childhood Soft Tissue SarcomaFor treatment of hemangiopericytoma (in infants and young children) and infantile fibrosarcoma
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.
- 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 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.
- A clinical trial of targeted drug therapy.
For treatment of clear cell sarcoma
Treatment of clear cell sarcoma will be surgery whenever possible. Other treatments may include the following:
- Radiation therapy after surgery.
- 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 desmoplastic small round cell tumor
Treatment of desmoplastic small round cell tumor may include surgery, chemotherapy (which may be given before surgery), and radiation therapy.
For treatment of extraosseous osteosarcoma
See the PDQ summary on Osteosarcoma and Malignant Fibrous Histiocytoma of Bone Treatment 1 for more information on the treatment of extraosseous osteosarcoma.
For treatment of malignant peripheral nerve sheath tumor
Treatment of malignant peripheral nerve sheath tumor will be surgery whenever possible. Other treatments may include the following:
- Radiation therapy after surgery.
- 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 plexiform histiocytic tumor
Treatment of plexiform histiocytic tumor is surgery to completely remove the tumor.
For treatment of synovial sarcoma
Treatment of synovial sarcoma may include the following:
- Surgery with or without chemotherapy.
- Chemotherapy.
- 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 undifferentiated soft tissue sarcoma
Treatment of undifferentiated soft tissue sarcoma may be within a clinical trial for patients with nonrhabdomyosarcomatous soft tissue sarcomas (NRSTS). This study includes surgery and/or radiation therapy with or without 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.
- 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 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 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.
- 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.
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 2. 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 3.
Metastatic Childhood Soft Tissue SarcomaTreatment of metastatic childhood soft tissue sarcoma may include the following:
- Combination chemotherapy, radiation therapy, and surgery to remove cancer that has spread to the lungs.
- Stereotactic radiation therapy or repeat surgery if several tumors appear or come back in 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 list of cancer clinical trials that are now accepting patients with metastatic 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 3.
Recurrent and Progressive Childhood Soft Tissue SarcomaTreatment 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 list of cancer clinical trials that are now accepting patients with recurrent childhood soft tissue sarcoma 5. 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 3.
Glossary Termsalveolar soft part sarcoma (al-VEE-oh-ler … sar-KOH-muh)A soft tissue tumor that is most common in older children and teenagers. It begins in the soft supporting tissue that connects and surrounds the organs and other tissues. Alveolar soft part sarcoma usually occurs in the legs, but can also occur in the arms, hands, head, or neck. It can cause the growth of new blood vessels that help the tumor grow and spread. Also called ASPS.angiosarcoma (AN-jee-oh-sar-KOH-muh) 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.antiestrogen (AN-tee-ES-truh-jin) A substance that keeps cells from making or using estrogen (a hormone that plays a role in 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. Also called estrogen blocker.blood clot (blud klot) 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.cancer (KAN-ser) A term for diseases in which abnormal cells divide without control and can invade nearby tissues. Cancer cells can also spread to other parts of the body through the blood and lymph systems. There are several main types of cancer. Carcinoma is a cancer that begins in the skin or in tissues that line or cover internal organs. Sarcoma is a cancer that begins in bone, cartilage, fat, muscle, blood vessels, or other connective or supportive tissue. Leukemia is a 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. Also called malignancy.chemotherapy (KEE-moh-THAYR-uh-pee) Treatment with drugs that kill cancer cells.clear cell (kleer sel) A type of cell that looks clear inside when viewed under a microscope.clinical trial (KLIH-nih-kul TRY-ul) 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.combination chemotherapy (KOM-bih-NAY-shun KEE-moh-THAYR-uh-pee) Treatment using more than one anticancer drug.desmoid tumor (DEZ-moyd TOO-mer) 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.desmoplastic small round cell tumor (DES-moh-PLAS-tik ... TOO-mer) A rare, aggressive cancer that usually affects young males and usually is located in the abdomen.disorder (dis-OR-der) In medicine, a disturbance of normal functioning of the mind or body. Disorders may be caused by genetic factors, disease, or trauma.external radiation therapy (...RAY-dee-AY-shun THAYR-uh-pee) 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.extraosseous (EK-struh-AH-see-us) Located outside of the bone.fibrosarcoma (FY-broh-sar-KOH-muh) A type of soft tissue sarcoma that begins in fibrous tissue, which holds bones, muscles, and other organs in place.grade (grayd) A description of a tumor based 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.granulocyte colony-stimulating factor (GRAN-yoo-loh-SITE KAH-luh-nee-STIM-yoo-LAY-ting FAK-ter) 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.hemangiopericytoma (hee-MAN-jee-oh-PAYR-ih-sy-TOH-muh) A type of cancer involving blood vessels and soft tissue.imatinib mesylate (ih-MA-tih-nib MEH-zih-layt) 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.internal radiation therapy (in-TER-nul RAY-dee-AY-shun THAYR-uh-pee) 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.liver (LIH-ver) A large organ located in the upper abdomen. The liver cleanses the blood and aids in digestion by secreting bile.lung (lung) One of a pair of organs in the chest that supplies the body with oxygen, and removes carbon dioxide from the body.lymphangiosarcoma (lim-FAN-jee-oh-sar-KOH-muh) A type of cancer that begins in the cells that line lymph vessels.malignant peripheral nerve sheath tumor (muh-LIG-nunt peh-RIH-feh-rul ... TOO-mer) 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.metastatic (meh-tuh-STA-tik) Having to do with metastasis, which is the spread of cancer from the primary site (place where it started) to other places in the body.nonsteroidal anti-inflammatory drug (NON-steh-ROY-dul AN-tee-in-FLA-muh-TOR-ee ...) A drug that decreases fever, swelling, pain, and redness. Also called NSAID.osteosarcoma (OS-tee-oh-sar-KOH-muh) A cancer of the bone that usually affects the large bones of the arm or leg. It occurs most commonly in young people and affects more males than females. Also called osteogenic sarcoma.PDQ PDQ is an online database developed and maintained by the National Cancer Institute. Designed to make the most current, credible, and accurate cancer information available to health professionals and the public, PDQ contains peer-reviewed summaries on cancer treatment, screening, prevention, genetics, complementary and alternative medicine, and supportive care; a registry of cancer clinical trials from around the world; and directories of physicians, professionals who provide genetics services, and organizations that provide cancer care. Most of this information, and more specific information about PDQ, can be found on the NCI's Web site at http://www.cancer.gov/cancertopics/pdq. Also called Physician Data Query.progressive disease (pruh-GREH-siv dih-ZEEZ) Cancer that is growing, spreading, or getting worse.radiation therapy (RAY-dee-AY-shun THAYR-uh-pee) 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.recurrent cancer (ree-KER-ent KAN-ser) 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.soft tissue sarcoma (…TIH-shoo sar-KOH-muh) A cancer that begins in the muscle, fat, fibrous tissue, blood vessels, or other supporting tissue of the body.stage (stayj) 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.stereotactic radiation therapy (STAYR-ee-oh-TAK-tik RAY-dee-AY-shun THAYR-uh-pee) A type of external radiation therapy that uses special equipment to position the patient and precisely deliver radiation to a tumor. The total dose of radiation is divided into several smaller doses given over several days. Stereotactic radiation therapy is used to treat brain tumors and other brain disorders. It is also being studied in the treatment of other types of cancer, such as lung cancer. Also called stereotactic external-beam radiation therapy and stereotaxic radiation therapy.surgery (SER-juh-ree) A procedure to remove or repair a part of the body or to find out whether disease is present. An operation.symptom (SIMP-tum) An indication that a person has a condition or disease. Some examples of symptoms are headache, fever, fatigue, nausea, vomiting, and pain.synovial sarcoma (sih-NOH-vee-ul sar-KOH-muh) A malignant tumor that develops in the synovial membrane of the joints.targeted therapy (TAR-geh-ted THAYR-uh-pee) A type of treatment that uses drugs or other substances, such as monoclonal antibodies, to identify and attack specific cancer cells. Targeted therapy may have fewer side effects than other types of cancer treatments.therapy (THAYR-uh-pee) Treatment.transplantation (tranz-plan-TAY-shun) 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).tumor (TOO-mer) 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 cancer), or malignant (cancer). Also called neoplasm.undifferentiated (un-DIH-feh-REN-shee-AY-ted) A term used to describe cells or tissues that do not have specialized ("mature") structures or functions. Undifferentiated cancer cells often grow and spread quickly.watchful waiting (WACH-ful WAY-ting) Closely watching a patient’s condition but not giving treatment unless symptoms appear or change. Watchful waiting is sometimes used in conditions that progress slowly. It is also used when the risks of treatment are greater than the possible benefits. During watchful waiting, patients may be given certain tests and exams. Watchful waiting is sometimes used in prostate cancer. It is a type of expectant management.white blood cell (hwite blud sel) A type of immune cell. Most white blood cells are made in the bone marrow and are found in the blood and lymph tissue. White blood cells help the body fight infections and other diseases. Granulocytes, monocytes, and lymphocytes are white blood cells. Also called leukocyte and WBC. |
