Disease Information
Solid Tumor: Non-Rhabdomyosarcoma Soft Tissue Sarcoma (NRSTS)
Alternate Names: NRSTS synovial sarcoma malignant peripheral nerve sheath tumor fibrosarcoma leiomyosarcoma, liposarcoma, epithelioid sarcoma, malignant fibrous histiocytoma, clear cell sarcoma, undifferentiated sarcoma, hemangiopericytoma, dermatofibrosarcoma
Definition
- Non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) are cancers that arise in the soft tissues of the body.
- Specific examples include synovial sarcoma, malignant peripheral nerve sheath tumor, fibrosarcoma, malignant fibrous histiocytoma, hemangiopericytoma, epithelioid sarcoma and liposarcoma.
- These cancers can develop virtually anywhere in the body, including the head and neck region, chest, abdomen, pelvis, arms and legs. They arise most commonly in the arms and legs.
Incidence
Tumors classified as NRSTS comprise about 4 percent of all cancers occurring in childhood.
Influencing Factors
- Adolescents and infants under 1 year of age are more likely than other children to develop NRSTS.
- Children in certain families with a genetic predisposition to cancer and those who have received radiation therapy in the past have a higher risk of developing NRSTS.
- Children and adolescents with type I neurofibromatosis are prone to develop malignant peripheral nerve sheath tumors.
Survival Rates
- Survival rates depend on the size of the tumor, its appearance under the microscope, the proportion of the tumor that can be removed surgically and whether the tumor has spread to other parts of the body.
Treatment Strategies
- Surgical removal of the tumor is the most effective treatment.
- Patients with tumor remaining after surgery may benefit from radiation therapy.
- Patients whose tumor cannot be surgically removed may receive chemotherapy and/or radiation therapy to shrink the tumor so that it can be removed.
- Certain patients with high-risk disease may benefit from chemotherapy.
Current Research
- A risk-based treatment approach is being tested in an effort to identify effective therapy while limiting the side effects of treatment.
- Laboratory studies focused on improving our understanding of these uncommon childhood cancers are ongoing.
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Research
