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

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    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.[4] (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.[5] This heterogeneous group of tumors includes neoplasms of:[6]

    • 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
    Rhabdomyosarcomas 710 466 364 350 41
    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 tumors a 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
    1 | 2 | 3 | 4 | 5 | 6
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