The commonest kidney cancer of childhood, and one of the great successes of modern oncology: more than 90% of children treated for it survive. The genetics that changes care is not on this page, and the page says which test finds it.
Start with the number that matters. Wilms tumour is a cancer of the kidney that occurs in young children, and more than 90% of children treated for it in high-income countries survive. It is one of the outcomes paediatric oncology changed most: within living memory it was usually fatal, and it is now usually not.
It affects roughly 1 in 10,000 children, most often diagnosed between the ages of 3 and 4. It is usually found as a painless swelling in the abdomen, often by a parent or during a routine examination.
Most Wilms tumours occur in children with no family history and no other condition. Genetics enters this page in two quite different ways, and confusing them is the mistake worth avoiding.
Predisposition, which is rare and acts. About 1 child in 10 with Wilms tumour has a recognised predisposition — changes in WT1, the imprinted region behind Beckwith-Wiedemann syndrome, and a number of others. These matter because they change what is done: a child known to be predisposed is offered regular ultrasound surveillance, which finds tumours smaller and earlier, and relatives can be assessed. That is a genuine clinical decision made from a genetic finding.
Common variants, which are the three on this page. A 2012 genome-wide study compared many children with the tumour against many without and found positions that differ slightly in frequency — near MYCN, and at two other loci. Each shifts risk by a small amount. None is a test, none is used in clinics, and carrying one says essentially nothing about an individual child.
The predisposition syndromes are not here. They are caused by rare changes and by imprinting faults that a scan for common variants does not see, and they are found by clinical genetic testing arranged through a paediatric oncology or genetics service — not by a consumer array and not by reading a file.
So if the question behind the search is whether a child is predisposed, this page cannot answer it and neither can any raw data file. The people who can are the clinical team, and the reason to ask them is concrete: a positive answer changes the surveillance schedule.
How it presents. Most often as an abdominal mass noticed without pain, sometimes with blood in the urine or raised blood pressure. Diagnosis is by imaging and, in most protocols, confirmed at surgery or biopsy.
Why survival is what it is. Wilms tumour was among the first cancers treated with combined surgery, chemotherapy and radiotherapy on a trial protocol, and successive international trials have spent decades reducing treatment for children who do well rather than only intensifying it for those who do not. Much of the modern work is about giving less, not more.
Predisposition syndromes worth naming. WT1-related conditions including WAGR and Denys-Drash, Beckwith-Wiedemann syndrome and other overgrowth conditions, and a small number of familial cases. Recognising one of these is what puts a child into surveillance.
The common variants in context. The 2012 study reported associations of the size typical for common variants in a rare disease. They contribute to understanding how these tumours arise; they do not identify which child will get one, and no clinical guideline uses them.
What a 23andMe/AncestryDNA export or raw VCF can and can't tell you about Wilms Tumour comes down to these specific, well-studied positions — not a diagnosis.
The studies behind these variants recruited participants from different ancestries — a result found in one population doesn't always transfer to another. Based on 3 of 3 linked studies with a resolved discovery ancestry.
Databases, guidelines and references
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Wilms Tumour. MyGeneLog™. https://www.mygenelog.com/conditions/wilms-tumour
Very little that helps today, and it is better to say so. The number worth holding on to is that more than 90% of children treated for Wilms tumour survive. The three variants here are research findings about risk across populations, not something that changes a treatment plan. Your child’s team is the right source for anything specific.
Usually not. Most children who develop it have no family history. About 1 in 10 have a recognised predisposition syndrome, and most of those changes arose new in that child rather than being passed down.
A clinical one can, and it is worth asking about, because a positive result changes care — predisposed children are offered regular ultrasound surveillance that finds tumours earlier. A consumer genotyping array cannot: the relevant changes are rare, and some are imprinting faults that this kind of test does not look for at all.
No. These shift risk by a small amount across large groups and are not used in any clinic. Wilms tumour is a disease of early childhood; a variant reported in an adult’s file has no bearing on them.
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