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Cancer Health Center

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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 tumors1313157866.5
Nerve sheath tumors1932741045
Other fibromatous neoplasms11120.1
Kaposi sarcoma120 12 0.3
Other specified soft tissue sarcomas19822051285638
Ewing tumor and Askin tumor of soft tissue222857814
pPNET of soft tissue211929422.4
Extrarenal rhabdoid tumor373831
Fibrohistiocytic tumorsa536917129312
Synovial sarcomas12391332048.3
Blood vessel tumors15711331.4
Osseous and chondromatous neoplasms of soft tissue159160.6
Alveolar soft parts sarcoma3719261
Miscellaneous soft tissue sarcomas161831352
Unspecified soft tissue sarcomas7058136 1639
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