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1. Sarcomas are a heterogeneous group of tumors that can occur throughout the body and encompass more than 50 subtypes with distinct histologic lines of differentiation.

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2. These rare tumors account for less than 1% of cancers in adults (estimated 10,000 cases per year in the United States) and represent 7% of cancers in children.

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3. Approximately two thirds of soft tissue sarcomas arise in the extremities; the remaining one third are distributed between the retroperitoneum, trunk, abdomen, and head and neck.

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4. The treatment algorithm for soft tissue sarcomas depends on tumor stage, site, and histology.

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5. Multimodality treatment including surgical resection, radiation therapy and, in selected cases, systemic chemotherapy, has been applied to patients with locally advanced, high-grade, extremity sarcomas.

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6. Overall 5-year survival rate for patients with all stages of soft tissue sarcoma is 50 to 60%.

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7. Of the patients who die of sarcoma, most will succumb to metastatic disease in the lungs, which 80% of the time occurs within 2 to 3 years of the initial diagnosis.

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8. Progress in the understanding of soft tissue sarcoma biology is crucial for the development of new therapeutic targets.

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Incidence

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Sarcomas are a heterogeneous group of tumors that arise predominantly from the embryonic mesoderm, but also can originate, as does the peripheral nervous system, from the ectoderm. In 2007, approximately 9220 new cases of soft tissue sarcoma were diagnosed in the United States with 3560 deaths attributable to disease.1 These rare tumors account for less than 1% of cancers in adults and represent 7% of cancers in children. Several distinct groups of sarcomas are recognized; soft tissue sarcomas, the largest of these groups, are the focus of this chapter. Other groups include bone sarcomas (osteosarcomas and chondrosarcomas), Ewing’s sarcomas, and peripheral primitive neuroectodermal tumors.

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Soft tissue sarcomas can occur throughout the body and encompass more than 50 histiotypes (Table 36-1) with distinct histologic lines of differentiation. The most common histologic types of soft tissue sarcoma in adults (excluding Kaposi’s sarcoma) are malignant fibrous histiocytoma (MFH, 28%), leiomyosarcoma (12%), liposarcoma (15%), synovial sarcoma (10%), and malignant peripheral nerve sheath tumors (6%).2 Rhabdomyosarcoma is the most common soft tissue sarcoma of childhood. Most primary soft tissue sarcomas originate in an extremity (50 to 60%); the next most common sites are the trunk, retroperitoneum, and head and neck.

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Table Graphic Jump Location
Table 36-1 Relative Frequency of Histologic Subtypes of Soft Tissue Sarcoma2 

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