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Genetic Predisposition to Wilms Tumour

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Renal Tumors of Childhood

Part of the book series: Pediatric Oncology ((PEDIATRICO))

Abstract

Wilms tumour is a primarily sporadic disease, with only 1–2 % of affected individuals having a relative with Wilms tumour. However, bilateral Wilms tumours occur in approximately 5 % of cases, and Wilms tumour has been reported in association with more than 50 different genetic disorders, pointing to an underlying predisposition in further individuals. There is conclusive evidence of an increased risk of Wilms tumour in only a small number of disorders, including familial Wilms tumour, the WT1-related syndromes, certain overgrowth disorders including Beckwith-Wiedemann syndrome and a small number of other cancer predisposition syndromes. The identification of the molecular defects that underlie these Wilms predisposition syndromes clarifies the risks of Wilms tumour risks and assists in the targeting of Wilms surveillance to those at increased risk. For example, in some disorders such as the 11p15-overgrowth disorders, it has emerged that only a subset of individuals are at increased risk of Wilms tumour. The discovery of further Wilms predisposition alleles is set to continue and will improve our ability to identify and manage those at increased risk of Wilms tumour.

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  1. Section of Cancer Genetics, Institute of Cancer Research, 15 Cotswold Road, Sutton, Surrey, SM2 5NG, UK

    Richard H. Scott & Nazneen Rahman

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  1. Department of Developmental Biology and Cancer, Institute of Child Health, University College London, London, United Kingdom

    Kathy Pritchard-Jones

  2. Children's National Medical Center Center for Cancer & Blood Disorders, Washington, District of Columbia, USA

    Jeffrey S. Dome

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Scott, R.H., Rahman, N. (2014). Genetic Predisposition to Wilms Tumour. In: Pritchard-Jones, K., Dome, J. (eds) Renal Tumors of Childhood. Pediatric Oncology. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-44003-2_2

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