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

Childhood soft tissue sarcoma is a disease in which malignant (cancer) cells form in soft tissues of the body.

Soft tissues of the body connect, support, and surround other body parts and organs. The soft tissues include the following:

  • Muscles.
  • Tendons (bands of tissue that connect muscles to bones).
  • Synovial tissues (tissues around joints).
  • Fat.
  • Blood vessels.
  • Lymph vessels.
  • Nerves.

Soft tissue sarcoma may be found anywhere in the body. In children, the tumors form most often in the arms, legs, or trunk (chest and abdomen).

There are many different types of soft tissue sarcomas.

The cells of each type of sarcoma look different under a microscope. The soft tissue tumors are grouped based on the type of soft tissue cell where they first formed.

See the following PDQ summaries for more information:

  • Childhood Rhabdomyosarcoma Treatment
  • Ewing Sarcoma Family of Tumors Treatment
  • Unusual Cancers of Childhood

This summary is about the following types of soft tissue sarcoma:

Fibrous (connective) tissue tumors

  • Desmoid tumor.
  • Dermatofibrosarcoma.
  • Fibrosarcoma.
  • Low-grade fibromyxoid sarcoma.

Fibrohistiocytic tumors

  • Malignant fibrous histiocytoma (MFH). This is also called undifferentiated pleomorphic sarcoma or spindle cell sarcoma. (See the PDQ summary on Osteosarcoma and Malignant Fibrous Histiocytoma of Bone Treatment for more information on MFH of bone.)
  • Plexiform histiocytic tumor.

Fat tissue tumors

  • Liposarcoma.

Smooth muscle tumors

  • Leiomyosarcoma.

Peripheral nervous system tumors

  • Malignant schwannoma (malignant peripheral nerve sheath tumor).

Bone and cartilage tumors

  • Extraosseous osteosarcoma.
  • Extraosseous myxoid chondrosarcoma.
  • Mesenchymal chondrosarcoma.


  • Angiomyolipoma.
  • Lymphangioleiomyomatosis.
  • Clear cell "sugar" tumor.

Tumors with more than one type of tissue

  • Malignant mesenchymoma.
  • Malignant Triton tumor.
  • Malignant ectomesenchymoma.

Tumors of unknown origin (the place where the tumor first formed is not known)

  • Alveolar soft part sarcoma.
  • Clear cell sarcoma (malignant melanoma of soft parts).
  • Desmoplastic small round cell tumor.
  • Epithelioid sarcoma.
  • Synovial sarcoma.
  • Undifferentiated soft tissue sarcoma.

Blood and lymph vessel tumors

  • Angiosarcoma.
  • Hemangioendothelioma.
  • Hemangiopericytoma.
  • Lymphangiosarcoma.

Besides rhabdomyosarcomas, the most common soft tissue sarcomas in children are in joint tissue, connective tissue, and nerve tissue.

Soft tissue sarcoma occurs in children and adults. Soft tissue sarcoma in children may respond differently to treatment, and may have a better outcome than soft tissue sarcoma in adults. (See the PDQ summary on Adult Soft Tissue Sarcoma Treatment for more information on treatment in adults.)

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