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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.[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 yAge 5–9 yAge 10–14 yAge 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 sarcomas1,1308101,1441,573 100
Fibrosarcomas, peripheral nerve, and other fibrous neoplasms15164132 19212
 Fibroblastic and myofibroblastic tumors131315786 6.5
 Nerve sheath tumors193274104 5
 Other fibromatous neoplasms1112 0.1
Kaposi sarcoma120 12 0.3
Other specified soft tissue sarcomas19822051285638
 Ewing tumor and Askin tumor of soft tissue22285781 4
 pPNET of soft tissue21192942 2.4
 Extrarenal rhabdoid tumor37383 1
 Liposarcomas562266 2
 Fibrohistiocytic tumorsa5369171293 12
 Leiomyosarcomas13192257 2.4
 Synovial sarcomas1239133204 8.3
 Blood vessel tumors1571133 1.4
 Osseous and chondromatous neoplasms of soft tissue15916 0.6
 Alveolar soft parts sarcoma371926 1
 Miscellaneous soft tissue sarcomas16183135 2
Unspecified soft tissue sarcomas7058136 1639
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