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• General Information About Childhood Soft Tissue Sarcoma
• Stages of Childhood Soft Tissue Sarcoma
• Recurrent and Progressive Childhood Soft Tissue Sarcoma
• Treatment Option Overview
• Treatment Options for Childhood Soft Tissue Sarcoma
• To Learn More About Childhood Soft Tissue Sarcoma

• Bone and Soft Tissue Oncology

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Treatment Options for 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 Sarcoma

For 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 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. 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.

Metastatic Childhood Soft Tissue Sarcoma

Treatment 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. 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.

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 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. General information about clinical trials is available from the NCI Web site.