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

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Treatment of sclerosing epithelioid fibrosarcoma may include the following:

  • Surgery to completely remove the tumor.

Skeletal Muscle Tumors

Rhabdomyosarcoma

See the PDQ summary on Childhood Rhabdomyosarcoma Treatment.

Smooth Muscle Tumors

Leiomyosarcoma

There is no standard treatment for leiomyosarcoma.

So-called Fibrohistiocytic Tumors

Plexiform fibrohistiocytic tumor

Treatment of plexiform fibrohistiocytic tumor may include the following:

  • Surgery to completely remove the tumor.

Undifferentiated pleomorphic sarcoma

There is no standard treatment for undifferentiated pleomorphic sarcoma.

Peripheral Nervous System Tumors

Malignant peripheral nerve sheath tumor

The treatment of malignant peripheral nerve sheath tumor may include the following:

  • Surgery to completely remove the tumor when possible.

It is not clear whether giving radiation therapy or chemotherapy after surgery improves the tumor's response to treatment.

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 if the tumor cannot be completely removed by surgery.
  • A clinical trial of targeted therapy.

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 may be given after surgery.

Desmoplastic small round cell tumor

There is no standard treatment for desmoplastic small round cell tumor. Treatment may include the following:

  • A combination of chemotherapy, surgery, and radiation therapy.

Epithelioid sarcoma

Treatment of epithelioid sarcoma may include the following:

  • Surgery to remove the tumor when possible.
  • Chemotherapy.
  • Radiation therapy.

Perivascular epithelioid cell tumors (PEComas)

Treatment of perivascular epithelioid cell tumors may include the following:

  • Surgery to remove the tumor.
  • A clinical trial of targeted therapy (mTOR 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.

Extraskeletal myxoid chondrosarcoma

Treatment of extraskeletal myxoid chondrosarcoma may include the following:

  • Surgery to remove the tumor when possible.
  • A clinical trial of targeted therapy.

Primitive neuroectodermal tumor (PNET)/extraskeletal Ewing tumor

See the PDQ summary on Ewing Sarcoma Treatment.

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