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29-04-2016 | Sarcoma | Book chapter | Article

20. Soft Tissue Sarcoma

Authors: MD, MPH Mary T. Austin, MD, FACS, FAAP Richard J. Andrassy

Publisher: Springer Berlin Heidelberg


Soft tissue sarcomas (STS) are a heterogeneous group of mesenchymal malignancies that account for 7.4 % of all pediatric malignancies. They are generally classified into two broad categories: nonrhabdomyosarcoma soft tissue sarcomas (NRSTS) and rhabdomyosarcomas (RMS) with RMS being the most common STS among children and adolescents. The epidemiology, clinical presentation and diagnosis of each tumor type as well as the staging work-up, treatment approach and anticipated outcomes will be discussed. Attention will be given to the surgical approach in the management of these tumors including a discussion of newer techniques such as sentinel lymph node biopsy. The following NRSTS histologies will be reviewed: alveolar soft part sarcoma, desmoplastic small round cell tumor (DSRCT), infantile and adult-type fibrosarcoma, fibrohistiocytic tumors, leiomyosarcoma, liposarcoma, malignant peripheral nerve sheath tumor (MPNST) and synovial sarcoma. A general overview of RMS will be given as well as a site based discussion of the disease.

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