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

    Table 1. Age Distribution of Soft Tissue Sarcomas (STSs) in Children Aged 0 to 19 Years (SEER 1975-2008) continued...

    Figure 1. The distribution of nonrhabdomyosarcomatous soft tissue sarcomas in children aged 0 to 19 years, as reported by the Surveillance Epidemiology and End Results program from 1975 to 2008.

    Nonrhabdomyosarcomatous STSs are more common in adolescents and adults,[6] and most of the information regarding treatment and natural history of the disease in younger patients has been based on adult studies.

    Risk Factors

    Some genetic and environmental factors have been associated with the development of nonrhabdomyosarcomatous STS:

    • Genetic factors:
      • Li-Fraumeni syndrome: Patients with Li-Fraumeni syndrome (usually due to heritable cancer-associated changes of the p53 tumor suppressor gene) have an increased risk of developing soft tissue tumors (mostly nonrhabdomyosarcomatous STSs), bone sarcomas, breast cancer, brain tumors, and acute leukemia.[5,9]
      • Neurofibromatosis type 1: Approximately 4% of patients with neurofibromatosis type 1 develop malignant peripheral nerve sheath tumors, which usually develop after a long latency; some patients develop multiple lesions.[10,11,12]
      • Familial adenomatous polyposis: Patients with familial adenomatous polyposis are at increased risk of developing desmoid tumors.[13]
      • Werner syndrome: Werner syndrome is characterized by spontaneous chromosomal instability, resulting in increased susceptibility to cancer and premature aging. An excess of STSs has been reported in patients with Werner syndrome.[14]
      • Retinoblastoma gene: Germline mutations of the retinoblastoma gene have been associated with an increased risk of developing STSs, particularly leiomyosarcoma.[15]
    • Environmental factors:
      • Radiation: Some nonrhabdomyosarcomatous STSs (particularly malignant fibrous histiocytoma) can develop within a previously irradiated site.[5,16]
      • Epstein-Barr virus infection in patients with AIDS: Some nonrhabdomyosarcomatous STSs (e.g., leiomyosarcoma) have been linked to Epstein-Barr virus infection in patients with AIDS.[5,17]

    Clinical Presentation

    Although nonrhabdomyosarcomatous STSs can develop in any part of the body, they arise most commonly in the trunk and extremities.[7,18,19] These neoplasms can present initially as an asymptomatic solid mass, or they may be symptomatic because of local invasion of adjacent anatomical structures.

    Systemic symptoms (e.g., fever, weight loss, and night sweats) are rare. Hypoglycemia and hypophosphatemic rickets have been reported in cases of hemangiopericytoma, whereas hyperglycemia has been noted in patients with fibrosarcoma of the lung.[20]

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