CION Cancer Clinics
VHL kidney surveillance and the 3 cm rule | CION Cancer Clinics
In VHL, small kidney tumours are usually watched with regular MRI scans and treated when the largest reaches about three centimetres. Below that size they very rarely spread, and waiting protects the kidneys from repeated surgery. This page explains the rule, what a scan can show, how often checks happen and what treatment looks like when the time comes. At CION Cancer Clinics, our oncologists plan screening and care for families with an inherited cancer syndrome, explained in plain words.
On this page
- What is the 3 cm rule in VHL?
- What can a VHL kidney scan show?
- How does kidney surveillance run through life?
- The words you will meet, in plain language
- What changes when a tumour reaches about three centimetres?
- Four things families worry about, and what is actually true
- What this page cannot tell you
- Common questions about VHL kidney surveillance
The short answer
What is the 3 cm rule in VHL?
In VHL, solid kidney tumours are usually watched with regular scans and treated when the largest one reaches about three centimetres across. Below that size, spread to other parts of the body is very rare. Waiting until then protects healthy kidney tissue without adding much danger.
Why not remove every tumour straight away?
VHL keeps making new cysts and tumours in both kidneys throughout life. Operating on each one as it appeared would mean many operations, scarring and a steady loss of kidney function. Some people treated that way would end up on dialysis. The rule spaces treatment out so that each operation counts.
What the rule protects
It protects the number of healthy kidney units you keep, and so your chance of avoiding dialysis in later life. It also keeps the number of operations over a lifetime as low as safely possible. When treatment is needed, the surgeon removes only the tumour and keeps the rest of the kidney wherever it can be done.
The rule is a guide used by experienced teams. Your team also weighs how fast a tumour is growing, where it sits and how well your kidneys work.On the scan
What can a VHL kidney scan show?
Most people with VHL have something on a kidney scan at some point. What matters is which of these four it is.
Simple cysts
Thin-walled pockets of fluid. They are very common in VHL and on their own usually need no treatment, only the routine scan.
Cysts with solid parts
A cyst with a thickened wall or a solid area inside it. The solid part can behave like a tumour, so it is measured and watched in the same way as one.
Small solid tumours
A kidney cancer of the clear cell type, still below the size where treatment is usually advised. Scans become more frequent so that growth can be tracked closely.
A tumour near the threshold
When the largest solid tumour nears about three centimetres, or is growing quickly, the team plans treatment.
- Kidney-sparing surgery
- Ablation, which destroys the tumour with heat or cold
- Occasionally a tablet to shrink it first
Not sure whether this applies to you?
Ask an oncologistOver the years
How does kidney surveillance run through life?
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From the mid-teens
An MRI of the abdomen is added to the VHL checks. It looks at the kidneys, the pancreas and the adrenal glands in one scan, often on the same day as the brain and spine MRI.
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While the kidneys look clear
The abdominal MRI is usually repeated every other year. Your blood creatinine is checked to follow kidney function.
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When a solid tumour appears
Scans move closer together, often every several months to a year, so that the team can see how quickly it is growing.
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As it nears the threshold
A urologist who knows VHL plans treatment. A blood or urine test for adrenal hormones is always done first, because a hidden adrenal tumour can make surgery dangerous.
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After treatment
Watching starts again. New tumours often appear elsewhere in the same or the other kidney, so the same rule is applied to them in turn.
On your report
The words you will meet, in plain language
- Clear cell renal cell carcinoma
- The type of kidney cancer that VHL causes. On a report it is often shortened to clear cell RCC.
- Solid lesion
- A lump made of tissue rather than fluid. The size of the largest solid lesion is what the 3 cm rule measures.
- Partial nephrectomy
- An operation that removes the tumour and a rim of normal tissue while keeping the rest of the kidney.
- Ablation
- Destroying a small tumour with heat or extreme cold through a needle, without open surgery.
- Creatinine and eGFR
- Blood measures of how well the kidneys filter. Your team follows them over the years to protect kidney function.
- Contrast
- A dye given into a vein during a scan to make tumours stand out. Your kidney function is checked before it is given.
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Side by side
What changes when a tumour reaches about three centimetres?
Commonly believed
Four things families worry about, and what is actually true
For most cancers that instinct is right. In VHL, small kidney tumours grow slowly and new ones keep appearing. Watching them until the right moment is the safer long-term plan, and it is what expert centres do.
Usually it is not. The other kidney will almost certainly develop tumours too. Keeping as much working kidney as possible is what keeps people off dialysis later.
Ultrasound can miss small solid tumours and cannot measure them as reliably, and the timing of treatment depends on accurate measurement. MRI is preferred for VHL surveillance, and it uses no radiation, which matters over decades of scans.
Most VHL kidney cysts are simple fluid pockets and stay harmless. The team measures the solid parts, and only those set the timing of treatment. A long list of cysts on a report can look frightening while meaning very little.
Being straight with you
What this page cannot tell you
It cannot tell you what your own scan shows or when your tumour should be treated. Measuring a VHL kidney well takes a radiologist and a urologist who see this condition often, comparing each scan with the last. What your specific variant means is a question for the counsellor who ordered the test.
The rule is not the whole decision
Some teams act a little earlier for a tumour growing fast, or one close to the main blood vessels of the kidney. Others wait a little longer when kidney function is already poor. Newer tablets that can shrink VHL tumours are changing the picture, though access and cost in India vary and studies are still maturing.
Who this does not apply to
The 3 cm rule was developed for people with VHL and a few other inherited kidney cancer conditions. It does not apply to a single kidney cancer found in someone without an inherited fault, which is planned differently. If you have tested negative for your family's VHL fault, you need no VHL kidney checks at all.
Questions we are asked
Common questions about VHL kidney surveillance
Is it safe to leave a kidney cancer inside me?
For small VHL kidney tumours, careful watching with regular scans is the accepted approach at expert centres. Tumours below about three centimetres very rarely spread. The key is that the scans actually happen on time, and are read by someone who compares them with the previous ones.
How often will I need a kidney scan?
While the kidneys look clear, usually every other year from the mid-teens. Once a solid tumour is found, the gap shortens so its growth can be tracked. Your team sets the interval for you, and it may change from one scan to the next. Put the next due date somewhere the whole family can see it.
Why MRI rather than CT?
People with VHL need abdominal scans for most of their lives. MRI uses no radiation, so repeated scans add no radiation dose. CT is sometimes used when MRI is not available or not suitable, and before surgery to map the blood vessels. Ask for the same type of scan each time where you can.
What operation will I have when the time comes?
Usually a partial nephrectomy, which removes the tumour and keeps the kidney. It can often be done through keyhole or robotic surgery. Small tumours in some positions can be treated with ablation through a needle instead. Nearby smaller tumours are often dealt with in the same operation.
Why is an adrenal test done before kidney surgery?
VHL can also cause a hormone-making adrenal tumour. If it has not been found, the stress of an operation can send blood pressure dangerously high. A blood or urine test before any planned surgery rules this out, or allows it to be treated first. Remind any new surgeon that you have VHL.
Is there a tablet for VHL kidney tumours?
Newer tablets that target the pathway VHL disrupts can shrink kidney and other VHL tumours and delay surgery in some people. They have side effects, including a low haemoglobin, and are not safe in pregnancy. Access and cost in India vary, so ask your oncologist whether one suits you.
Will I end up on dialysis?
Avoiding it is the whole point of the 3 cm rule and of kidney-sparing surgery, and most people managed this way keep enough kidney function. The risk rises with each operation, so the number of procedures is kept as low as safely possible. Your creatinine and eGFR are followed over the years to spot any decline.
Can the kidney scans be done near my home?
The MRI can often be done at a good centre closer to home, if the images are shared with your VHL team. Consistency matters, because measurements are compared scan to scan. Ask for the images on a disc or a link, not only the printed report.
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.
Dr. C. Raghavendra Reddy
MBBS(Gold Medal), DNB(General Medicine), DM(Medical Oncology)(Gold Medal)
Dr. Bharati Devi Gorantla
MBBS, MD(General Medicine), DM(Medical Oncology)(Adyar,Chennai), ECMO, MRCP SCE(UK)
Dr. Owais Mohammed
MBBS, MD (General Medicine), DrNB (Medical Oncology), ECMO, MRCP SCE (Medical Oncology) (UK)
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Our centres in and around Hyderabad
Addressed by landmark, because that is how this city navigates. One helpline books a consultation at any of these centres, and your team will tell you where counselling and testing take place.
Sources
- GeneReviews (NCBI) — Von Hippel-Lindau Syndrome
- National Cancer Institute — Genetics of Kidney Cancer (Renal Cell Cancer) (PDQ)
- MedlinePlus Genetics — Von Hippel-Lindau syndrome
- Cancer.Net (ASCO) — Von Hippel-Lindau Syndrome
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.
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