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WT1, CDKN1C and the other Wilms tumour genes: what they do | CION Cancer Clinics
Several genes guide how a baby's kidneys form and keep kidney cells from growing out of turn. The best known are WT1 and CDKN1C, which sits in a growth region of chromosome 11. When one is faulty from birth, a child has a higher chance of Wilms tumour. Most children with Wilms tumour carry no such fault. This page explains what these genes do and why it matters. 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 do the Wilms tumour genes actually do?
- Which genes are linked to Wilms tumour?
- How can a fault present at birth lead to a kidney tumour?
- The words you will meet, in plain language
- When is a Wilms tumour more likely to be inherited?
- Four things families tell us, and what is actually true
- What this page cannot tell you
- Common questions about the Wilms tumour genes
The short answer
What do the Wilms tumour genes actually do?
These genes guide how a baby's kidneys form in the womb, and some of them act as brakes on cell growth. When one of them is faulty from birth, a few kidney cells may never finish maturing. A small number of those cells can later grow into a Wilms tumour, a kidney cancer of early childhood.
WT1, the kidney-building gene
WT1 switches other genes on and off while the kidneys and the sex organs are forming. After birth it keeps working in the tiny filters of the kidney. A fault in WT1 can therefore cause kidney disease and differences in genital development, as well as a raised chance of Wilms tumour.
CDKN1C and the growth region on chromosome 11
CDKN1C is a brake on cell division. It sits in a small region of chromosome 11 where genes are switched on or off depending on which parent they came from. Changes in this region cause Beckwith-Wiedemann syndrome, an overgrowth condition. In that condition Wilms tumour is one of several childhood tumours that doctors watch for.
Most Wilms tumours are not inherited. The faults usually arise in the kidney alone and cannot be passed on.The genes on a report
Which genes are linked to Wilms tumour?
No single gene explains inherited Wilms tumour. These are the ones a genetic report is most likely to name.
WT1
A master switch for kidney and genital development. Faults can be tiny spelling changes or a missing piece of chromosome that takes WT1 with it.
Linked conditions
- WAGR syndrome, which also affects the eyes
- Denys-Drash syndrome
- Frasier syndrome
The chromosome 11 growth region
This region holds CDKN1C and two growth genes called IGF2 and H19. Most changes here are switching errors that arise by chance and are not inherited. Spelling faults in CDKN1C are the exception. They are more often passed down, usually through the mother.
Rarer genes
Several other genes carry a smaller or less well measured risk. Each has its own pattern, and some matter more for other cancers than for the kidney.
Examples include
- DIS3L2, behind Perlman syndrome
- DICER1, REST and TRIM28
- The genes behind Bloom syndrome and Li-Fraumeni syndrome
Why the exact gene matters
The gene, and often the exact change, sets how high the risk is. It also decides whether the kidneys, eyes or genitals need watching alongside the scans, and how long those checks continue.
Not sure whether this applies to you?
Ask an oncologistFrom gene to tumour
How can a fault present at birth lead to a kidney tumour?
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The kidneys form in the womb
Early kidney cells mature into the filtering units a child will use for life. WT1 and its partner genes steer that change, step by step.
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A fault can leave some cells unfinished
Small clusters of immature cells can stay behind in the kidney after birth. They are called nephrogenic rests. Most shrink away on their own and never cause harm.
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A second change happens in one cell
For a gene like WT1, the child starts with one faulty copy in every cell. If the working copy is damaged in one of those unfinished cells, that cell loses its brake.
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A tumour can form in early childhood
Wilms tumour mostly appears in the first few years of life. This is why screening for predisposed children is focused on early childhood and then stops.
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Why the risk differs so much
Some conditions carry a high chance and others a modest one. The syndrome, the gene and the exact change all matter, and the counsellor weighs all three when planning checks.
On your report
The words you will meet, in plain language
- Wilms tumour
- A kidney cancer of young children, also called nephroblastoma. Most children are treated successfully, often with surgery and chemotherapy.
- Nephrogenic rests
- Small patches of immature kidney cells left over from before birth. Many disappear. A few can grow into a tumour.
- Germline
- Present in every cell from birth, and so able to be passed on. A fault found only in the tumour is called somatic.
- Imprinting
- A natural system that switches some genes on or off depending on whether they came from the mother or the father.
- Methylation
- Chemical tags that act as the on and off switches for imprinted genes. Errors in these tags cause most Beckwith-Wiedemann syndrome.
- Mosaic
- A change present in only some of the body's cells. A blood test can miss it, so another sample may be needed.
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Side by side
When is a Wilms tumour more likely to be inherited?
More likely inherited
More likely not inherited
Tumours in both kidneys
One tumour in one kidney
Diagnosed unusually young
Diagnosed at the usual age
Overgrowth, a missing iris, or kidney or genital differences
No other health differences
A relative who also had Wilms tumour
No family history of it
Commonly believed
Four things families tell us, and what is actually true
"If my child has a Wilms gene fault, cancer is certain."
It raises the chance. It does not settle the matter. Many children with a predisposing fault never develop a tumour, and regular scans are designed to find any tumour while it is still small.
"Wilms tumour runs in families, so my other children will get it."
Only a small share of Wilms tumours are inherited. Most begin with changes inside one kidney that no brother or sister can share. A counsellor can tell you which group your child is in.
"Beckwith-Wiedemann syndrome is always passed down."
Most cases come from switching errors that happen by chance around conception. The inherited form, often a CDKN1C fault, is the less common one, and testing tells the two apart.
"A normal blood test means there is no fault."
Some changes are present in only part of the body. In Beckwith-Wiedemann syndrome especially, blood can look normal while skin or tumour tissue shows the change. A negative result is read in that light.
Being straight with you
What this page cannot tell you
It cannot tell you whether your child carries a fault, or which one. That is answered by a genetic counsellor or clinical geneticist who has examined your child, looked at the tumour report and drawn the family tree. Some features, like overgrowth on one side of the body, are easy to miss without a trained eye.
It cannot interpret a report you are holding
The same gene can carry very different risks depending on the exact change. What your child's specific result means is a question for the counsellor who ordered the test. Searching the variant online usually adds worry without adding clarity.
Who this does not apply to
Most children with Wilms tumour have a single tumour, no other health differences and no family history. For them, an inherited fault is unlikely, though many teams still offer counselling. Testing the tumour itself to guide treatment is a different test, covered on our targeted therapy pages.
If you are unsure whether your child's history fits, describe it to the helpline and someone will tell you honestly whether a referral is worth making.Questions we are asked
Common questions about the Wilms tumour genes
Is Wilms tumour inherited?
Usually not. Most Wilms tumours start with changes that arise inside one kidney and are not in the rest of the body. A smaller group of children carry a fault from birth, sometimes as part of a syndrome. A genetic assessment is how a family finds out which group they belong to.
What is the difference between WT1 and CDKN1C?
WT1 guides how the kidneys and genitals form, so its faults can affect kidney function and development too. CDKN1C is a growth brake in an imprinted region of chromosome 11, and its faults cause Beckwith-Wiedemann syndrome. The two lead to different checks and different family patterns.
Can a parent carry a fault without ever having had cancer?
Yes. A parent can carry a WT1 or CDKN1C fault and stay well. With CDKN1C, whether a child is affected depends partly on which parent passed it on, so a healthy father can carry it silently. Testing parents is how this is sorted out.
Does every child with Wilms tumour need a genetic test?
Not every child needs a full test, but many teams now offer every family a conversation. Testing is most useful when both kidneys are affected, the child is very young, or there are other features such as overgrowth, an eye difference or genital differences.
What is WAGR syndrome?
A condition caused by a missing piece of chromosome 11 that removes WT1 and a nearby eye gene together. Children often have no iris in the eye, differences in the genitals and developmental delay, along with a high chance of Wilms tumour. It is usually noticed from the eyes soon after birth.
Why does it matter which parent the change came from?
Some genes in the chromosome 11 region work only from the mother's copy, and others only from the father's. A CDKN1C fault usually causes Beckwith-Wiedemann syndrome only when a child inherits it from their mother. Your counsellor will explain what that means for each branch of the family.
Does a gene result change my child's treatment?
It can. If both kidneys are at risk, surgeons may try to keep as much healthy kidney as possible. A WT1 fault also means kidney function and blood pressure are watched for longer. The treating oncologist decides this with the genetics team.
Where do we start if a doctor has mentioned these genes?
Ask for a referral to a genetic counsellor or clinical geneticist, and bring the tumour report and any scan reports. Write down any other health differences your child has. Call the CION helpline if you are not sure who to approach, and someone will point you to the right clinic.
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
- MedlinePlus Genetics — WT1 gene
- MedlinePlus Genetics — CDKN1C gene
- MedlinePlus Genetics — Wilms tumor
- National Cancer Institute — Wilms Tumor and Other Childhood Kidney Tumors Treatment (PDQ) – Health Professional 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
Wilms tumour genes: which cancers and what riskHow high the risk is for each gene and syndromeBeckwith-Wiedemann syndrome and tumour screeningThe overgrowth condition linked to CDKN1CAbdominal ultrasound screening in predisposed childrenWhat the regular scans involveTesting the family for the Wilms tumour genesWhich relatives to test, and in what order
Talk to us
Has a doctor mentioned a Wilms tumour gene?
Tell us what the doctor said and what the reports show so far. We will help you reach a genetics team who can explain it and plan the next steps. One helpline serves every CION centre.