Childhood Soft Tissue Sarcoma Treatment (PDQ®): Treatment - Health Professional Information [NCI] - General Information
Rhabdomyosarcoma, a tumor of striated muscle, is the most common STS in children aged 0 to 14 years and accounts for 50% of tumors in this age group. (Refer to the PDQ summary on Childhood Rhabdomyosarcoma Treatment for more information.) The remaining STSs are commonly referred to as nonrhabdomyosarcomatous STSs and account for about 3% of all childhood tumors. This heterogeneous group of tumors includes neoplasms of:
- Connective tissue (e.g., desmoid fibromatosis, liposarcoma).
- Peripheral nervous system (e.g., malignant peripheral nerve sheath tumor).
- Smooth muscle (e.g., leiomyosarcoma).
- Vascular tissue (blood and lymphatic vessels, e.g., angiosarcoma).
In children, synovial sarcoma, fibrosarcoma, fibrohistiocytic tumors, and malignant peripheral nerve sheath tumors predominate.[7,8] The distribution of STSs by histology and age, based on the Surveillance Epidemiology and End Results (SEER) information from 1975 to 2008, is depicted in Table 1. The distribution of histologic types by age is shown in Figure 1.
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|