Sarcoma Epidemiology and Etiology: Potential Environmental and Genetic Factors

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Sarcomas are a heterogeneous group of tumors that may have many etiologies. The incidence of histologic subtypes differs significantly between children and adults. The increase in incidence may be due to improved registry systems, diagnostic tools, and pathologic definitions. Environmental causes may contribute to increased incidence. Genetic alternations may play a role in sarcoma development. As a result of rapidly evolving genomic and proteomic technologies, increased knowledge of the oncogenic mechanisms underlying sarcomagenesis is being generated. Understanding the mechanisms involved in sarcomagenesis is rudimentary. Insight into the molecular basis of sarcoma inception, proliferation, and dissemination hopefully will lead to more effective therapies.

Section snippets

Epidemiology

Sarcomas are a diverse group of malignant tumors that arise predominantly from the embryonic mesoderm and can be categorized as tumors arising primarily from the bone (eg, osteosarcoma and Ewing's sarcoma [ES]) and those that arise from soft tissues. The World Health Organization has defined approximately 50 soft tissue sarcoma (STS) histologic subtypes [1]. There is overlap, however, between certain tumors and the differences between subtypes are not always distinct. STSs are subcategorized

Etiology

Most STSs are etiologically indeterminate; however, several etiologic factors are identified, which can be divided into environmental and host/genetic considerations (Box 1).

Summary

Sarcomas are a markedly heterogeneous group of tumors that may have many etiologies. The incidence of different histologic subtypes differs significantly between children and adults. There is an increase in the incidence of sarcoma in the United States that may be the result of improved registry systems, diagnostic tools, and pathologic definitions rather than reflecting a true increase of the disease. Various environmental etiologies, however, also may contribute to a true increased sarcoma

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