Abstract
It is now generally accepted that cancer is caused by an accumulation of genetic mutations. However, only a small proportion of cancer-associated gene mutations are inherited (i.e. are germline mutations); the majority of gene mutations in cancer cells are acquired (somatic mutations) and are secondary to environmental carcinogens such as radiation, cigarette smoking or chemicals. Almost all human cancers occur in both sporadic and inherited forms (Hodgson and Maher 1992), and it appears that mutations in a restricted number of genes are involved in the genesis of both sporadic and inherited cancers. Consequently, the identification of the genetic mutations responsible for familial cancers has become an important step in understanding the molecular pathology of human carcinogenesis. In familial cancer syndromes, although an inherited germline mutation accounts for the cancer predisposition, this alone is not sufficient to produce a tumour and additional acquired mutations are also necessary. In this chapter the molecular pathology of Wilms’ tumour and renal cell carcinoma is discussed with particular emphasis on how the investigation of rare inherited cancer syndromes is contributing towards understanding the molecular genetics of these tumours.
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Maher, E.R. (1994). Molecular Genetics of Wilms’ Tumour and Renal Cell Carcinoma. In: Neal, D.E. (eds) Tumours in Urology. Springer, London. https://doi.org/10.1007/978-1-4471-2086-5_13
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DOI: https://doi.org/10.1007/978-1-4471-2086-5_13
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