Question: What are the treatments for Childhood Soft Tissue Sarcoma ?
Answer: Key Points - There are different types of treatment for patients with childhood soft tissue sarcoma. - 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 for childhood soft tissue sarcoma may cause side effects. - Eight types of standard treatment are used: - Surgery - Radiation therapy - Chemotherapy - Observation - Hormone therapy - Nonsteroidal anti-inflammatory drugs - Targeted therapy - Immunotherapy - New types of treatment are being tested in clinical trials. - Gene therapy - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. 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. Treatment for childhood soft tissue sarcoma may cause side effects. For information about side effects that begin during treatment for cancer, see our Side Effects page. Side effects from cancer treatment that begin 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.) Eight 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. This is called neoadjuvant therapy. 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. - Mohs surgery: A surgical procedure used to treat cancer in the skin. Individual layers of cancer tissue are removed and checked under a microscope one at a time until all cancer tissue has been removed. This type of surgery is used to treat dermatofibrosarcoma protuberans. It is also called Mohs micrographic surgery. - Hepatectomy: Surgery to remove all or part of the liver. 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 hepatectomy and 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. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. Certain ways of giving radiation therapy can help keep radiation from damaging nearby healthy tissue. This type of radiation therapy may include the following: - Stereotactic body radiation therapy: Stereotactic body radiation therapy is a type of external radiation therapy. Special equipment is used to place the patient in the same position for each radiation treatment. Once a day for several days, a radiation machine aims a larger than usual dose of radiation directly at the tumor. By having the patient in the same position for each treatment, there is less damage to nearby healthy tissue. 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 and whether the tumor was completely removed by surgery. External and internal radiation therapy are used to treat childhood soft tissue sarcoma. 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 of cancer being treated. Most types of soft tissue sarcoma do not respond to treatment with chemotherapy. See Drugs Approved for Soft Tissue Sarcoma for more information. Observation Observation is closely monitoring a patients condition without giving any treatment until signs or symptoms appear or change. Observation may be done when: - Complete removal of the tumor is not possible. - No other treatments are available. - The tumor is not likely to damage any vital organs. 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), such as tamoxifen, may be used to treat desmoid-type fibromatosis. 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 desmoid-type fibromatosis, an NSAID called sulindac may be used to help block the growth of cancer cells. Targeted therapy Targeted therapy is a type of treatment that uses drugs or other substances to attack cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation do. Kinase inhibitors are a type of targeted therapy that block an enzyme called kinase (a type of protein). There are different types of kinases in the body that have different actions. - ALK inhibitors may stop the cancer from growing and spreading: - Crizotinib may be used to treat inflammatory myofibroblastic tumor. - Tyrosine kinase inhibitors (TKIs) block signals needed for tumors to grow: - Imatinib is used to treat dermatofibrosarcoma protuberans. - Pazopanib may be used to treat recurrent and progressive soft tissue sarcoma. It is being studied for many types of newly diagnosed soft tissue sarcoma. - Sorafenib may be used to treat desmoid-type fibromatosis. New types of tyrosine kinase inhibitors are being studied such as LOXO-101 and entrectinib for infantile fibrosarcoma. Other types of targeted therapy are being studied in clinical trials, including the following: - mTOR inhibitors are a type of targeted therapy that stops the protein that helps cells divide and survive. mTOR inhibitors are being studied to treat perivascular epithelioid cell tumors (PEComas) and epithelioid hemangioendothelioma. Sirolimus is a type of mTOR inhibitor therapy. - Angiogenesis inhibitors are a type of targeted therapy that prevent the growth of new blood vessels needed for tumors to grow. Angiogenesis inhibitors, such as cediranib, sunitinib, and thalidomide are being studied to treat alveolar soft part sarcoma and epithelioid hemangioendothelioma. Bevacizumab is being studied for blood vessel tumors. - Histone methyltransferase (HMT) inhibitors are targeted therapy drugs that work inside cancer cells and block signals needed for tumors to grow. HMT inhibitors are being studied for the treatment of epithelioid sarcoma, malignant peripheral nerve sheath tumor, extrarenal (extracranial) rhabdoid tumor, extraskeletal myxoid chondrosarcoma, and synovial sarcoma. - Heat-shock protein inhibitors block certain proteins that protect tumor cells and help them grow. Ganetespib is a heat shock protein inhibitor being studied in combination with the mTOR inhibitor sirolimus for malignant peripheral nerve sheath tumors that cannot be removed by surgery. - Antibody-drug conjugates are made up of a monoclonal antibody attached to a drug. The monoclonal antibody binds to specific proteins or receptors found on certain cells, including cancer cells. The drug enters these cells and kills them without harming other cells. Lorvotuzumab mertansine is an antibody-drug conjugate being studied for the treatment of rhabdomyosarcoma, malignant peripheral nerve sheath tumor, and synovial sarcoma. See Drugs Approved for Soft Tissue Sarcoma for more information. Immunotherapy Immunotherapy is a treatment that uses the patients immune system to fight disease. Substances made by the body or made in a laboratory are used to boost, direct, or restore the bodys natural defenses against disease. Interferon is a type of immunotherapy used to treat epithelioid hemangioendothelioma. It interferes with the division of tumor cells and can slow tumor growth. 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. 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. - Radiation therapy before or after surgery. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. Bone and Cartilage Tumors Extraskeletal mesenchymal chondrosarcoma Treatment of extraskeletal mesenchymal chondrosarcoma may include the following: - Surgery to completely remove the tumor. Radiation therapy may be given before and/or after surgery. - Chemotherapy followed by surgery. Chemotherapy with or without radiation therapy is given after surgery. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. Extraskeletal osteosarcoma Treatment of extraskeletal osteosarcoma may include the following: - Surgery to completely remove the tumor, followed by chemotherapy. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. See the PDQ summary on Osteosarcoma and Malignant Fibrous Histiocytoma of Bone Treatment for more information. Fibrous (Connective) Tissue Tumors Desmoid-type fibromatosis Treatment of desmoid-type fibromatosis may include the following: - Surgery to completely remove the tumor. Treatment before surgery may include the following: - Observation. - Chemotherapy. - Radiation therapy. - Antiestrogen drug therapy. - Nonsteroidal anti-inflammatory drug (NSAID) therapy. If the tumor is not completely removed by surgery, treatment may include the following: - Observation, if other treatment options are not possible. - Radiation therapy. - Radiation therapy or chemotherapy for tumors that cannot be removed by surgery. - A clinical trial of targeted therapy. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. Treatment of desmoid-type fibromatosis that has come back may include the following: - Observation and possibly surgery at a later time. - Chemotherapy. Dermatofibrosarcoma protuberans Treatment of dermatofibrosarcoma protuberans may include the following: - Surgery to completely remove the tumor when possible. This may include Mohs surgery. - Radiation therapy before or after surgery. - Targeted therapy (imatinib) if the tumor cannot be removed or has come back. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. Fibrosarcoma Infantile fibrosarcoma Treatment of infantile fibrosarcoma may include the following: - Surgery to remove the tumor when possible, followed by observation. - Surgery followed by chemotherapy. - Chemotherapy to shrink the tumor, followed by surgery. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. - A clinical trial of targeted therapy (tyrosine kinase inhibitor). Adult-type fibrosarcoma Treatment of adult-type fibrosarcoma may include the following: - Surgery to completely remove the tumor when possible. Inflammatory myofibroblastic tumor Treatment of inflammatory myofibroblastic tumor may include the following: - Surgery to completely remove the tumor when possible. - Chemotherapy. - Steroid therapy. - Nonsteroidal anti-inflammatory drug (NSAID) therapy. - Targeted therapy (ALK inhibitors). - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. Low-grade fibromyxoid sarcoma Treatment of low-grade fibromyxoid sarcoma may include the following: - Surgery to completely remove the tumor. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. Myxofibrosarcoma Treatment of myxofibrosarcoma may include the following: - Surgery to completely remove the tumor. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. Sclerosing epithelioid fibrosarcoma Treatment of sclerosing epithelioid fibrosarcoma may include the following: - Surgery to completely remove the tumor. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. Skeletal Muscle Tumors Rhabdomyosarcoma See the PDQ summary on Childhood Rhabdomyosarcoma Treatment. Smooth Muscle Tumors Leiomyosarcoma Treatment of leiomyosarcoma may include the following: - Chemotherapy. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. So-called Fibrohistiocytic Tumors Plexiform fibrohistiocytic tumor Treatment of plexiform fibrohistiocytic tumor may include the following: - Surgery to completely remove the tumor. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. Peripheral Nervous System Tumors Ectomesenchymoma Treatment of ectomesenchymoma may include the following: - Surgery and chemotherapy. - Radiation therapy. Malignant peripheral nerve sheath tumor Treatment of malignant peripheral nerve sheath tumor may include the following: - Surgery to completely remove the tumor when possible. - Radiation therapy before or after surgery. - Chemotherapy, for tumors that cannot be removed by surgery. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. - A clinical trial of targeted therapy, for tumors that cannot be removed by surgery. - A clinical trial of targeted therapy (histone methyltransferase inhibitor). - A clinical trial of an antibody-drug conjugate. It is not clear whether giving radiation therapy or chemotherapy after surgery improves the tumor's response to treatment. Malignant triton tumor Malignant triton tumors may be treated the same as rhabdomyosarcomas and include surgery, chemotherapy, or radiation therapy. A regimen of targeted therapy, radiation therapy, and surgery with or without chemotherapy is being studied. Pericytic (Perivascular) Tumors Infantile hemangiopericytoma Treatment of infantile hemangiopericytoma may include the following: - Chemotherapy. Infantile myofibromatosis Treatment of infantile myofibromatosis may include the following: - Combination chemotherapy. 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 before or after surgery, if the tumor cannot be completely removed by surgery. - Targeted therapy (angiogenesis inhibitor). - A clinical trial of targeted therapy (angiogenesis inhibitor) for children. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. 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 before or after surgery. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. Desmoplastic small round cell tumor There is no standard treatment for desmoplastic small round cell tumor. Treatment may include the following: - Surgery to completely remove the tumor when possible. - Chemotherapy followed by surgery. - Radiation therapy. Epithelioid sarcoma Treatment of epithelioid sarcoma may include the following: - Surgery to remove the tumor when possible. - Chemotherapy before or after surgery. - Radiation therapy before or after surgery. - A clinical trial of targeted therapy (histone methyltransferase inhibitor). 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. - A clinical trial of targeted therapy (histone methyltransferase inhibitor). Extraskeletal myxoid chondrosarcoma Treatment of extraskeletal myxoid chondrosarcoma may include the following: - Surgery to remove the tumor when possible. - Radiation therapy. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. - A clinical trial of targeted therapy (histone methyltransferase inhibitor). Perivascular epithelioid cell tumors (PEComas) Treatment of perivascular epithelioid cell tumors may include the following: - Surgery to remove the tumor. - Observation followed by surgery. - Targeted therapy (mTOR inhibitor), for tumors that have certain gene changes and cannot be removed by surgery. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. 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: - Chemotherapy. - Surgery. Radiation therapy and/or chemotherapy may be given before or after surgery. - A clinical trial of gene therapy. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. - A clinical trial of targeted therapy (histone methyltransferase inhibitor). - A clinical trial of an antibody-drug conjugate. Undifferentiated/unclassified sarcoma These tumors include undifferentiated pleomorphic sarcoma /malignant fibrous histiocytoma (high-grade). There is no standard treatment for these tumors. Treatment may include the following: - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. See the PDQ summary on Osteosarcoma and Malignant Fibrous Histiocytoma of Bone Treatment for information about the treatment of malignant fibrous histiocytoma of bone. Blood Vessel Tumors Angiosarcoma of soft tissue Treatment of angiosarcoma may include the following: - Surgery to completely remove the tumor. - A combination of surgery, chemotherapy, and radiation therapy for angiosarcomas that have spread. - Targeted therapy (bevacizumab) and chemotherapy for angiosarcomas that began as infantile hemangiomas. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. Epithelioid hemangioendothelioma Treatment of epithelioid hemangioendothelioma may include the following: - Surgery to remove the tumor when possible. - Immunotherapy (interferon) and targeted therapy (thalidomide, sorafenib, pazopanib, sirolimus) for tumors that are likely to spread. - Chemotherapy. - Total hepatectomy and liver transplant when the tumor is in the liver. Metastatic Childhood Soft Tissue Sarcoma Treatment of childhood soft tissue sarcoma that has spread to other parts of the body at diagnosis may include the following: - Chemotherapy and radiation therapy. Surgery may be done to remove tumors that have spread to the lung. - Stereotactic body radiation therapy for tumors that have spread to the lung. For treatment of specific tumor types, see the Treatment Options for Childhood Soft Tissue Sarcoma section. Check the list of NCI-supported 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 website. Recurrent and Progressive Childhood Soft Tissue Sarcoma Treatment of recurrent or progressive childhood soft tissue sarcoma may include the following: - Surgery to remove cancer that has come back where it first formed or that has spread to the lung. - 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. - Chemotherapy. - Targeted therapy (tyrosine kinase inhibitor). - A clinical trial of a new chemotherapy regimen. Check the list of NCI-supported 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 website.