Rare Kidney Cancer In Children Caused By Genetic Mutation

Wilms' tumor--a rare kidney cancer that occurs in children--is the result of a genetic mutation in a gene called REST, researchers believe.

Researchers at The Institute of Cancer Research, London, identified mutations in the REST gene in 16 children with Wilms tumor--a rare health problem that's estimated to affect about 1 in 10,000 children. 

"We hope our findings will stimulate research into why and how these REST mutations, which all cluster in a particular part of the gene, cause cancer," said Professor Nazneen Rahman, Head of Genetics and Epidemiology at The Institute of Cancer Research, London, and Head of Cancer Genetics at The Royal Marsden NHS Foundation Trust, in a news release. 

Researchers predict that due to the clustering of cases, the REST mutations are responsible for 10 percent of familial Wilms' tumors. In this study, nine of the children were the only members of the family to develop Wilms' tumour, but in four families, more than one child had developed the cancer, as well. 

"Our findings are also of immediate value to families, who now have an explanation for why their child got cancer. Moreover, we can now do a simple blood test to see which children in the family are at risk of cancer and may benefit from cancer screening, and which have not inherited the mutation and so are not at increased risk of cancer. This kind of information is really valuable for the families of children with cancer."

The research is part of the Factors Associated with Childhood Tumours (FACT) study, which is uncovering genetic causes of childhood cancers and has participants from more than 5,000 families. The FACT study is funded by the Wellcome Trust and the Rosetrees Trust, and the REST project involved international collaborators including from Baylor College of Medicine in Texas, US.

The study is published in the journal Nature Genetics.

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