Treatment Options for Childhood Soft Tissue Sarcoma


Nonmetastatic Childhood Soft Tissue Sarcoma

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.

Metastatic Childhood Soft Tissue Sarcoma

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).

Recurrent and Progressive Childhood Soft Tissue Sarcoma

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.

Medications used to help treat Soft Tissue Sarcoma:

• Adriamycin (Doxorubicin Hydrochloride)
• Gleevec (Imatinib Mesylate)
• Sutent (Sunitinib Malate)