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Wilms tumour predisposition genes: which cancers, and how much risk | CION Cancer Clinics
Several different genes can raise a child's chance of Wilms tumour, a kidney cancer of early childhood. The main ones are WT1 and changes in a region of chromosome 11 that includes CDKN1C. Each carries its own pattern of risk, and for most the risk is highest before school age. This page sets out which cancers each gene is linked to and why the size of the risk varies. At CION Cancer Clinics, our oncologists explain what a gene result means for you and your family, and plan the checks that follow.
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Medically reviewed by Dr. Naresh GunduConsultant Medical Oncologist · MBBS, DNB (Internal Medicine), DM (Medical Oncology, AIIMS) · last reviewed September 2026, next review due September 2027
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On this page
- What does carrying a Wilms tumour gene fault mean?
- Which genes raise Wilms tumour risk, and which other cancers go with them?
- How is a child's risk level worked out?
- The words you will meet, in plain language
- Which pictures usually mean higher risk, and which lower
- Four things parents tell us about Wilms tumour genes, and what is true
- What this page cannot tell you
- Common questions about Wilms tumour gene risks
The short answer
What does carrying a Wilms tumour gene fault mean?
It means a child's chance of Wilms tumour, a kidney cancer of early childhood, is higher than usual. Depending on the gene, the chance of a liver tumour, a nerve tumour or kidney disease may be raised too. Most children with Wilms tumour do not have an inherited fault at all. Only a small minority have a recognised syndrome or gene change behind it.
Why risk is mostly a question for early childhood
Wilms tumour grows from leftover cells of the developing kidney. Most cases are found in children under school age, and the chance falls steeply as the kidneys finish maturing. For most of these genes, the tumour risk to an adult carrier is low, although some genes carry other risks that last longer.
Why the size of the risk varies so much
Different genes, and different changes within one gene, carry very different levels of risk. Published figures come from small groups of children, often collected through specialist centres that see the more severe cases. That is why your team will talk in terms of higher and lower risk rather than exact numbers.
A gene fault raises the chance of a tumour. It does not make one certain.The main genes
Which genes raise Wilms tumour risk, and which other cancers go with them?
These are the groups your counsellor is most likely to mention. Each has a different pattern beyond the kidney.
WT1
A gene that guides kidney and genital development. Faults can cause Wilms tumour alongside kidney disease and differences in genital development.
Linked conditions
- WAGR syndrome, with missing iris in the eye
- Denys-Drash syndrome, with early kidney disease
- Frasier syndrome, with a raised gonad tumour risk
The chromosome 11p15 region and CDKN1C
Changes here cause Beckwith-Wiedemann syndrome and some cases of one-sided overgrowth. Depending on the exact change, Wilms tumour, hepatoblastoma of the liver or neuroblastoma of nerve tissue may be raised.
Rarer genes in Wilms families
Genes such as TRIM28, REST, CTR9 and DIS3L2 have been found in families where Wilms tumour recurs. DIS3L2 causes Perlman syndrome, a rare overgrowth condition. Each is uncommon, and knowledge is still growing.
Wider cancer syndromes
DICER1 syndrome, Li-Fraumeni syndrome and a few others list Wilms tumour among many possible cancers. Here the wider syndrome, not the kidney, usually shapes the whole plan.
Not sure whether this applies to you?
Ask an oncologistStep by step
How is a child's risk level worked out?
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The gene or region is identified
A blood test, and sometimes a tissue test, finds which gene or chromosome region is involved. For BWS, the exact type of change also matters.
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Physical signs are recorded
Missing iris, overgrowth, kidney disease or genital differences all point to particular groups and help place the child's risk.
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The family history is drawn out
A counsellor asks about kidney tumours, kidney failure and other childhood cancers on both sides of the family.
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The team places the child in a higher or lower group
Guidelines offer regular screening when a child's risk is judged to be meaningfully above that of other children. Below that point, careful watching by parents and doctors is usually enough.
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The estimate is revisited as the child grows
New signs or a new family diagnosis can shift the picture. The plan is reviewed at each visit.
On your child's report
The words you will meet, in plain language
- Wilms tumour
- A kidney cancer of young children, also called nephroblastoma. It usually responds well to treatment.
- Predisposition
- A raised chance of developing a condition, not the condition itself.
- Germline
- Present in every cell from birth, and so able to be passed on. A change found only in the tumour is called somatic.
- Nephrogenic rests
- Small patches of immature kidney tissue left over from development. Some can turn into Wilms tumour.
- Bilateral
- Affecting both kidneys. Tumours on both sides make an inherited cause more likely.
- Gonadoblastoma
- A tumour of the ovary or testis tissue, linked to some WT1 conditions.
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Side by side
Which pictures usually mean higher risk, and which lower
Usually higher Wilms risk
Usually lower Wilms risk
WAGR, with the WT1 gene missing
Missing iris from a change that spares WT1
Denys-Drash syndrome
Frasier syndrome
BWS from an extra chemical tag at IC1
BWS from a lost chemical tag at IC2
Tumours in both kidneys at diagnosis
One tumour, with no other signs
A young child with a known family fault
An adult carrier past childhood
Commonly believed
Four things parents tell us about Wilms tumour genes, and what is true
"Our child had Wilms tumour, so it must be in our genes."
Most Wilms tumours are not inherited. Testing is usually offered when there are tumours in both kidneys, other physical signs, or more than one affected relative.
"All these genes carry the same risk."
They differ widely. Some carry a high chance of Wilms tumour, others a small one. Some matter more for the liver, nerves or kidney function than for Wilms tumour itself.
"An adult carrier needs kidney scans for life."
For most of these genes, Wilms tumour risk falls sharply after early childhood. Adults may still need kidney function checks or other care, depending on the gene.
"If one child has it, every child will."
It depends on the cause. Many cases are new in the child. When a parent carries the fault, each child has a separate chance, and testing can check.
Being straight with you
What this page cannot tell you
It cannot give your child a personal risk figure. That depends on the exact gene change, the physical signs and the family history, weighed together by a clinical geneticist. What your child's specific variant means is a question for the counsellor who ordered the test.
Where the evidence is thin
Many of these conditions are rare, and the rarer genes have been described in only a handful of families. Almost all the data come from outside India. Risk estimates may shift as more children are studied, so ask how you will be told if guidance changes.
Who this does not apply to
Most children treated for Wilms tumour, with a tumour in one kidney and no other signs or family pattern, do not need genetic testing. Tumour testing to guide treatment is a separate matter, handled by the treating team, and is covered under targeted therapy rather than here.
If your child has been treated for Wilms tumour, ask the treating team whether a genetics referral was considered.Questions we are asked
Common questions about Wilms tumour gene risks
Is Wilms tumour hereditary?
Usually not. Most cases arise by chance in one child. A small share are linked to an inherited gene fault or a syndrome, and even then a parent may not carry it, because the change can be new in the child.
Which signs suggest an inherited cause?
Tumours in both kidneys, a very young age at diagnosis, a missing iris, overgrowth, differences in genital development, early kidney disease, or another relative with Wilms tumour. Any of these is a reason to ask about a genetics referral.
Does a WT1 fault affect the kidneys beyond cancer?
It can. Some WT1 conditions cause kidney disease that leads to protein in the urine and, over time, reduced kidney function. Blood pressure and urine checks are often part of long-term care, separate from tumour screening.
Can an adult carrier develop Wilms tumour?
It is rare. Wilms tumour is overwhelmingly a cancer of young children. An adult carrier's main questions are usually about their own kidney health, any other cancers linked to the gene, and their children's risk.
What is the chance of passing it on?
For gene faults that follow the usual dominant pattern, each child has a one in two chance of inheriting it. BWS causes that are not inherited carry a much lower chance. Your counsellor will explain which applies to your family.
Should brothers and sisters be tested?
If a fault has been found and a parent carries it, young siblings are usually offered testing, since screening would start in early childhood. A sibling who tests negative needs no extra scans.
Does the risk differ if the child is a boy or a girl?
For Wilms tumour itself, not in any way that changes screening. Some WT1 conditions affect genital development differently, and the gonad tumour risk is looked at according to the child's chromosomes.
Where do we start?
Ask your child's oncologist or paediatrician for a referral to a clinical geneticist. Bring any reports and a family history covering kidney problems and childhood cancers. Call the CION helpline if you are unsure where to begin.
Your Specialists
Meet CION's oncologists. Bring your family history or genetic report to them.
Our medical oncologists see people with a strong family history of cancer, arrange genetic counselling and testing where it fits, and plan the checks that follow.
Medical Oncologist
Dr. C. Raghavendra Reddy
MBBS(Gold Medal), DNB(General Medicine), DM(Medical Oncology)(Gold Medal)
Medical Oncologist
Dr. Bharati Devi Gorantla
MBBS, MD(General Medicine), DM(Medical Oncology)(Adyar,Chennai), ECMO, MRCP SCE(UK)
Medical Oncologist
Dr. Owais Mohammed
MBBS, MD (General Medicine), DrNB (Medical Oncology), ECMO, MRCP SCE (Medical Oncology) (UK)
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Sources
- GeneReviews (NCBI Bookshelf) — Wilms Tumor Predisposition
- MedlinePlus Genetics — WT1 gene
- MedlinePlus Genetics — WAGR syndrome
- National Cancer Institute — Wilms Tumor and Other Childhood Kidney Tumors Treatment (PDQ) - Patient Version
This page is general information, not a prescription. Do not change or stop any treatment based on what you read here. If anything is worrying you, contact your own treating team — or call our helpline and we will help you reach the right specialist.
Keep reading
Related pages
Beckwith-Wiedemann syndrome and tumour screeningThe most common overgrowth cause, explainedSurveillance for Wilms tumour gene carriersHow often scans run, and when they stopWilms tumour genes positive: what happens nextThe first steps after a positive resultTesting the family for Wilms tumour genesWhich relatives should be offered a test
Talk to us
Wondering whether your child's Wilms tumour could be inherited?
Tell us about your child's diagnosis and your family history. We will tell you honestly whether a genetics referral is worth making, and arrange it if it is. One helpline serves every CION centre.