Medical Oncologist
CION Cancer Clinics
Von Hippel-Lindau disease, organ by organ | CION Cancer Clinics
Von Hippel-Lindau disease can cause growths in the eyes, brain and spinal cord, kidneys, adrenal glands, pancreas and inner ear. Most are not cancer, and the main cancer risk sits in the kidneys. No one develops problems everywhere. This page walks through each organ, what VHL can do there, how it is checked, and why everyone with the fault is watched in every organ. At CION Cancer Clinics, our oncologists plan screening and care for families with an inherited cancer syndrome, explained in plain words.
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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
- Which parts of the body can VHL affect?
- What VHL can do in each part of the body
- How does one team keep track of so many organs?
- The words you will meet, in plain language
- Which check looks at which organ?
- Four things families tell us, and what is actually true
- What this page cannot tell you
- Common questions about VHL and the organs it affects
The short answer
Which parts of the body can VHL affect?
Von Hippel-Lindau disease, or VHL, can affect the eyes, the brain and spinal cord, the kidneys, the adrenal glands, the pancreas and the inner ear. Most growths it causes are not cancer. The kidneys are where the main cancer risk lies.
VHL runs in families. Each child of a person with VHL has a one in two chance of inheriting the fault. Some people are the first in their family, with a new fault that neither parent carries, so a blank family history does not rule it out.
Why one gene reaches so many organs
The VHL gene helps cells sense how much oxygen they have. When it is faulty, a cell behaves as though it is short of oxygen and signals for new blood vessels to grow. That is why so many VHL growths are rich in blood vessels, and why they can appear in any organ where that signal matters.
No two people have the same pattern
A person with VHL will not develop problems in every organ. Even within one family, a parent might have kidney tumours while a child has eye and brain growths. The exact gene fault shapes some of this, and chance does the rest. This is why everyone who carries the fault is checked in every organ, not only where the family has had trouble.
VHL is a lifelong condition, but most of its growths can be found early and managed well.Organ by organ
What VHL can do in each part of the body
Grouped the way a VHL clinic thinks about them: what each growth is, and why it matters.
Eyes, brain and spine
Blood-vessel tumours called haemangioblastomas. In the retina they are often the earliest sign of VHL, sometimes in childhood. In the brain and spine they can press on nerves.
Can cause
- Blurred or lost vision
- Headache, unsteadiness, vomiting
- Numbness or weakness in the limbs
Kidneys
Cysts and a type of kidney cancer called clear cell renal cell carcinoma. These are often several, in both kidneys, and usually cause no symptoms until large. Regular scans let small tumours be watched and treated at the right size, saving as much kidney as possible.
Adrenal glands
Phaeochromocytomas, tumours that release adrenaline in bursts. They are usually not cancer but can be dangerous during surgery, childbirth or a sudden surge. They run more in some VHL families than others.
Can cause
- High blood pressure
- Pounding heartbeat and sweating
- Episodes of severe headache
Pancreas, ear and reproductive organs
Pancreatic cysts are common and mostly harmless. Pancreatic neuroendocrine tumours are less common and need watching. Tumours of the inner ear can affect hearing and balance. Small harmless cysts can form near the testes, or rarely near the womb.
Not sure whether this applies to you?
Ask an oncologistHow a VHL clinic works
How does one team keep track of so many organs?
One coordinator
One doctor, often a medical oncologist or clinical geneticist, holds the whole schedule. Specialists for each organ report back to them, so nothing falls between departments.
Checks from childhood
Eye examinations, blood pressure and a general check begin in early childhood. Children with VHL are seen every year even when they feel completely well.
Scans added with age
Brain and spine MRI, and MRI of the abdomen for the kidneys, pancreas and adrenal glands, are added around the teenage years. Hearing tests are added too.
One visit where possible
Tests are grouped into a single visit when they can be. For a family travelling from a district, this saves days of travel and missed work.
On your reports
The words you will meet, in plain language
- Haemangioblastoma
- A non-cancerous tumour made of blood vessels, found in the retina, brain or spinal cord.
- Renal cell carcinoma
- Kidney cancer. The clear cell type is the one linked to VHL, and in VHL it often grows slowly.
- Phaeochromocytoma
- An adrenal gland tumour that releases adrenaline. It can raise blood pressure sharply.
- Metanephrines
- Breakdown products of adrenaline, measured in blood or urine to look for a phaeochromocytoma.
- Endolymphatic sac tumour
- A slow-growing tumour of the inner ear that can affect hearing and balance.
- Pancreatic neuroendocrine tumour
- A solid growth in the pancreas that is watched closely and sometimes removed.
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Side by side
Which check looks at which organ?
Organ
How it is usually checked
Retina
Dilated eye examination by an eye specialist
Brain and spinal cord
MRI of the brain and whole spine
Kidneys and pancreas
MRI of the abdomen, or ultrasound in children
Adrenal glands
Blood pressure and a blood or urine hormone test
Inner ear
Hearing test, and a scan if hearing changes
Commonly believed
Four things families tell us, and what is actually true
"VHL is a kind of kidney cancer."
VHL is an inherited condition that can cause growths in several organs. Kidney cancer is one part of it, and the most important one to watch, but many people with VHL are first diagnosed through their eyes or brain.
"My father only had kidney problems, so I only need kidney scans."
The pattern can differ between parent and child, even with the same fault. Everyone who carries it follows the full programme for every organ.
"Cysts in the kidney or pancreas mean cancer."
Most VHL cysts are harmless and stay that way. Solid growths are what the team watches more closely.
"No symptoms means nothing is growing."
Most VHL growths cause no symptoms while they are small. That is exactly when they are easiest to manage, and why checks continue when you feel well.
Being straight with you
What this page cannot tell you
It cannot tell you which organs will be affected in you or your child. Nobody can predict that fully. The exact VHL fault gives some clues, for instance about the adrenal glands, but chance plays a large part.
It also cannot tell you when a particular growth will need treatment. That depends on its size, where it sits and how fast it is changing. The specialist for that organ decides, together with the doctor who coordinates your care.
It cannot interpret your result
What your specific variant means is a question for the counsellor who ordered the test. Some faults are linked to a higher chance of phaeochromocytoma, and your counsellor will explain whether yours is one of them.
Who this does not apply to
Relatives who tested negative for the family's VHL fault do not need this programme. Most people with a single kidney cyst, a pancreatic cyst or high blood pressure do not have VHL at all. And testing a kidney tumour for faults inside the tumour is a different question, handled under targeted therapy.
Counselling can be arranged in Telugu, and relatives are welcome to attend.Questions we are asked
Common questions about VHL and the organs it affects
Is VHL a type of cancer?
No. It is an inherited condition that raises the chance of growths in several organs. Most are not cancer. The main cancer risk is in the kidneys, which is why kidney scans form a central part of the lifelong programme.
Which organ is usually affected first?
Often the eyes, sometimes in childhood, which is why eye checks start early. Adrenal tumours can also appear young in some families. Kidney and pancreatic growths tend to come later, in adult life.
Will I definitely develop kidney cancer?
Not definitely. The chance is much higher than in the general population, and many people with VHL develop kidney tumours at some point. Regular scans let them be treated while small, often keeping most of the kidney working.
Why does the surgeon need to know about VHL before any operation?
An undetected phaeochromocytoma can cause a dangerous rise in blood pressure under anaesthesia. Before any planned surgery, including a caesarean, a person with VHL should be checked for one. Tell every surgeon and anaesthetist about VHL.
Can VHL affect fertility?
Cysts near the testes are usually harmless, though rarely they can affect fertility if both sides are involved. Most people with VHL can have children. Each child has a one in two chance of inheriting the fault, which a counsellor can discuss.
Are VHL Type 1 and Type 2 different diseases?
They are the same condition, grouped by whether a family is prone to phaeochromocytoma. Type 2 families are. The label guides how closely the adrenal glands are watched, but everyone still follows checks for every organ.
Can a healthy lifestyle prevent VHL growths?
No lifestyle change stops VHL growths from forming. Not smoking protects the kidneys and blood vessels, and good blood pressure control helps. The most effective step is keeping every scheduled check.
How do we get the whole programme organised?
Ask for one doctor to coordinate every organ's checks and hold the full record. Bring your genetic report and all past scans. Call the CION helpline if you need help setting this up.
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)
Medical Oncologist
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Sources
- GeneReviews (NCBI) — Von Hippel-Lindau Syndrome
- MedlinePlus Genetics — Von Hippel-Lindau syndrome
- MedlinePlus Genetics — VHL gene
- National Cancer Institute — Genetics of Kidney Cancer (Renal Cell Cancer) (PDQ)
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
Kidney surveillance and the size ruleHow kidney tumours are watched and when they are treatedPhaeochromocytoma screening in VHLThe adrenal check that matters before any surgeryBrain and spine surveillance in VHLWhat the MRI looks for and when it startsVHL Type 1 and Type 2 explainedWhy some families are watched more closely for adrenal tumours
Talk to us
Need help pulling every VHL check into one plan?
Bring your genetic result and your past scans. We will help you find one team to coordinate every organ's checks. One helpline serves every CION centre.