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)
|Age <5 y||Age 5–9 y||Age 10–14 y||Age 15–19 y||% of the Total Number of STS Cases <20 y|
|pPNET = peripheral primitive neuroectodermal tumors; SEER = Surveillance Epidemiology and End Results.|
|a Dermatofibrosarcoma accounts for 75% of these cases.|
|All soft tissue and other extraosseous sarcomas||1,130||810||1,144||1,573 ||100|
|Fibrosarcomas, peripheral nerve, and other fibrous neoplasms||151||64||132 ||192||12|
| ||Fibroblastic and myofibroblastic tumors||131||31||57||86|| ||6.5|
| ||Nerve sheath tumors||19||32||74||104|| ||5|
| ||Other fibromatous neoplasms||1||1||1||2|| ||0.1|
|Kaposi sarcoma||1||2||0 ||12 ||0.3|
|Other specified soft tissue sarcomas||198||220||512||856||38|
| ||Ewing tumor and Askin tumor of soft tissue||22||28||57||81|| ||4|
| ||pPNET of soft tissue||21||19||29||42|| ||2.4|
| ||Extrarenal rhabdoid tumor||37||3||8||3|| ||1|
| ||Liposarcomas||5||6||22||66|| ||2|
| ||Fibrohistiocytic tumorsa||53||69||171||293|| ||12|
| ||Leiomyosarcomas||13||19||22||57|| ||2.4|
| ||Synovial sarcomas||12||39||133||204|| ||8.3|
| ||Blood vessel tumors||15||7||11||33|| ||1.4|
| ||Osseous and chondromatous neoplasms of soft tissue||1||5||9||16|| ||0.6|
| ||Alveolar soft parts sarcoma||3||7||19||26|| ||1|
| ||Miscellaneous soft tissue sarcomas||16||18||31||35|| ||2|
|Unspecified soft tissue sarcomas||70||58||136 ||163||9|
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, and most of the information regarding treatment and natural history of the disease in younger patients has been based on adult studies.
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.
- 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.
- Retinoblastoma gene: Germline mutations of the retinoblastoma gene have been associated with an increased risk of developing STSs, particularly leiomyosarcoma.
- 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]