Etiology and Predisposition
This chapter briefly summarizes the most updated information on soft tissue tumors’ etiology and predisposition.
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Etiology and Predisposition
As it occurs for most human neoplasms, the etiology of most soft tissue tumors is basically unknown, and the vast majority of them are sporadic. In a minority of cases, the following risk factors have been identified: (a) Among physical agents, ionizing radiations are a well-known risk factor. For instance, radiotherapy increases by 10–50 times the risk of some soft tissue sarcomas (e.g., angiosarcoma and undifferentiated pleomorphic sarcoma) as well as the risk of osteosarcoma; of note, the interval between radiation exposure and tumor development is averagely 10–15 years; noteworthy, the combination with chemotherapy appears to shorten the time to radiation-induced sarcoma. (b) Some chemical agents (e.g., herbicides such as phenoxyacetic acid, wood preservatives containing chlorophenol, contrast medium thorotrast/thorium oxide, industrial chemicals such as vinyl chloride and arsenic) are also recognized carcinogens associated with increased risk of soft tissue sarcomas (with special regard to angiosarcoma). (c) Biological agents (i.e., viruses) have been linked to the development of specific soft tissue sarcomas: the best example is the tight relationship between human herpesvirus 8 (HHV8) and Kaposi sarcoma. (d) Chronic lymphedema (following lymphadenectomy but also filarial infection and congenital primary lymphedema) is associated with an increased risk of lymphangiosarcoma, a condition known as Stewart-Treves syndrome. (e) In a minority of cases, soft tissue tumors run in families where they contribute to the phenotype of cancer predisposition syndromes, such as hereditary retinoblastoma (associated with the development of different types of sarcomas), Li-Fraumeni syndrome (multiple types of sarcomas), Werner syndrome (undifferentiated pleomorphic sarcoma as well as other sarcomas), Gardner syndrome (desmoid tumor), Carney-Stratakis syndrome (GIST and paraganglioma), Gorlin syndrome (rhabdomyosarcoma), tuberous sclerosis (PEComas), and so on. These hereditary syndromes are generally due to germline high penetrance © The Editor(s) (if applicable) and The Author(s), under exclusive license to Springer Nature Switzerland AG 2021 S. Mocellin, Soft Tissue Tumors, https://doi.org/10.1007/978-3-030-58710-9_3
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3 Etiology and Predisposition
inactivation of tumor suppressor genes (e.g., RB1 in hereditary retinoblastoma, TP53 in Li-Fraumeni syndrome, NF1 in neurofibromatosis, and so on) or activation of oncogenes (e.g., KIT in familial GIST). The details of these as well as many other conditions predisposing to soft tissue tumor development are described in the sections dedicated to single tumors (in the second part of this book). Due to their rarity, sarcomas have not been the target of large epidemiological studies. A recently launched multicentric population-based case-control French study (ETIOSARC) will investigate the role of lifestyle and environmental and occupational factors in the occurrence of sarcomas among adults. As regards the molecular etiopathogenesis of sporadic
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