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Childhood Soft Tissue Sarcoma Treatment (PDQ®): Treatment - Patient Information [NCI] - Treatment Option Overview


Most types of soft tissue sarcoma do not respond to treatment with chemotherapy.

See Drugs Approved for Soft Tissue Sarcoma for more information.

Watchful waiting

Watchful waiting is closely monitoring a patient's condition without giving any treatment until signs or symptoms appear or change. Watchful waiting may be done when:

  • Complete removal of the tumor is not possible.
  • No other treatments are available.
  • The tumor does not place any vital organs in danger.

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.

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.

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.

Tyrosine kinase inhibitors (TKIs) are a type of targeted therapy that blocks signals needed for tumors to grow:

  • Imatinib is a tyrosine kinase inhibitor used in the treatment of dermatofibrosarcoma protuberans.

Other types of targeted therapy are being studied in clinical trials, including the following:

  • Pazopanib is a tyrosine kinase inhibitor being studied in the treatment of recurrent or progressive soft tissue sarcoma.
  • 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 and sunitinib, are being studied to treat alveolar soft part sarcoma and blood vessel tumors.
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