What is childhood soft tissue sarcoma?
Childhood soft tissue sarcoma is a disease in which cancer (malignant) cells
begin growing in soft tissue in the body. The soft tissues include muscles,
tendons (bands of fiber that connect muscles to bones), fibrous (connective)
tissues, fat, blood vessels, nerves, and synovial tissues (tissues around
joints). Soft tissues connect, support, and surround other body parts and
Soft tissue sarcomas are rare in children and adolescents. If a patient has
symptoms of a soft tissue sarcoma, the doctor may order x-rays and other tests.
The doctor may also cut out a small piece of tissue and have it looked at under
the microscope to see if there are any cancer cells. This is called a biopsy.
If cancer cells are found, the doctor may remove as much tumor as safely possible, along with some healthy tissue around it, during the same surgery. Some soft tissue sarcomas may be completely removed by surgery.
There are many different kinds of soft tissue sarcoma, depending on the soft
tissue where the cancer begins. Rhabdomyosarcoma is the most common type of
childhood soft tissue sarcoma. It begins in muscles around the bone and can be
found anywhere in the body. (Refer to the PDQ summaries on Childhood
Rhabdomyosarcoma Treatment and
Adult Soft Tissue Sarcoma Treatment for more information.) The soft tissue
sarcomas that affect young patients include tumors of the smooth muscle,
connective tissue, blood and lymphatic vessels, and the peripheral nervous
Soft tissue sarcomas may develop in any part of the body, but in young patients,
they are most commonly found in the trunk, arms, and legs. The first symptom
may be a solid mass or lump. If the mass interferes with a function of the
body, it may cause other symptoms. Soft tissue sarcoma rarely causes fever,
weight loss, or night sweats.
Soft tissue sarcoma is more likely to develop in people who have specific
genetic conditions, such as Li-Fraumeni syndrome, who have previously received
radiation therapy, or who have the Epstein-Barr virus with acquired immune
deficiency syndrome (AIDS).
Soft tissue sarcomas are classified according to the type of soft tissue they
resemble. The types of soft tissue sarcoma include:
- TUMORS OF FIBROUS (CONNECTIVE) TISSUE:
- desmoid tumor
- FIBROHISTIOCYTIC TUMORS:
- malignant fibrous histiocytoma
- FAT TISSUE TUMORS:
- SMOOTH MUSCLE TUMORS:
- BLOOD AND LYMPH VESSEL TUMORS:
- PERIPHERAL NERVOUS SYSTEM TUMORS:
- malignant schwannoma (malignant peripheral nerve sheath tumor)
- BONE AND CARTILAGE TUMORS:
- extraosseous osteosarcoma
- extraosseous myxoid chondrosarcoma
- extraosseous mesenchymal chondrosarcoma
- TUMORS WITH MORE THAN ONE TYPE OF TISSUE:
- malignant mesenchymoma
- malignant Triton tumor
- malignant ectomesenchymoma
- TUMORS OF UNKNOWN ORIGIN:
- alveolar soft part sarcoma
- epithelioid sarcoma
- clear cell sarcoma (malignant melanoma of soft parts [MMSP])
- synovial sarcoma
- desmoplastic small round cell tumor
The chance of recovery (prognosis) and choice of treatment depend on the type,
location, and stage of the tumor and the age, size, stage of development, and
general health of the patient.