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Adrenal screening in VHL: finding a phaeochromocytoma early | CION Cancer Clinics
VHL can cause a phaeochromocytoma, an adrenal tumour that releases stress hormones and can send blood pressure dangerously high. A simple yearly blood or urine test finds most of these tumours before they cause harm. This page explains the symptoms, how screening works, why the test must come before any surgery or pregnancy, and what treatment involves. 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
- Why does a VHL carrier need adrenal screening?
- What symptoms can a phaeochromocytoma cause?
- How does adrenal screening work over the years?
- The words you will meet, in plain language
- Four things people assume, and what is actually true
- What this page cannot tell you
- Common questions about adrenal screening in VHL
The short answer
Why does a VHL carrier need adrenal screening?
Because VHL can cause a phaeochromocytoma, a tumour of the adrenal gland that releases stress hormones. It can stay silent for years, then send blood pressure dangerously high, especially during an operation, a pregnancy or childbirth. A simple yearly blood or urine test finds most of these tumours long before that happens.
What the adrenal glands do
You have two adrenal glands, one sitting on top of each kidney. Their inner part makes adrenaline and noradrenaline, the hormones behind the racing heart you feel when frightened. A phaeochromocytoma makes these hormones without control, sometimes in sudden bursts.
Which families are most affected
Some VHL families have many phaeochromocytomas and others have very few, depending on the type of variant in the family. Because that pattern is not perfectly predictable, screening is advised for every carrier. Similar tumours can also form outside the adrenal glands, along nerves in the abdomen or neck, and these are called paragangliomas.
Screening is about safety as much as cancer. Most of these tumours do not spread. The danger is what their hormones can do to blood pressure.Signs of too much hormone
What symptoms can a phaeochromocytoma cause?
Many people have none, which is why the test matters. When symptoms come, they often arrive in attacks that pass within an hour or less.
Pounding headaches
Sudden, severe headaches that throb with the heartbeat, often during an attack of other symptoms. They can be set off by exercise, bending, straining or pressure on the abdomen.
A racing heart
A heartbeat you can feel pounding or racing in your chest, without exercise or fright to explain it. Some people also feel chest tightness or a sense of dread.
Heavy sweating and pallor
Drenching sweats and a pale, anxious look, sometimes with shaking or a feeling of panic. These attacks are easily mistaken for anxiety, which delays the diagnosis in many people.
High blood pressure
Especially in a child, a young adult, or anyone whose blood pressure is hard to control on tablets.
- Blood pressure that jumps up and down
- Dizziness on standing
Not sure whether this applies to you?
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Two things that cannot wait
A sudden attack of severe headache with a pounding heart, sweating or chest pain in someone with VHL is an emergency. Go to the nearest emergency department the same day and say that you have VHL. And never go into planned surgery, or into labour, without an adrenal test first. If you are pregnant and have not been tested, tell your obstetrician today.
From first test to treatment
How does adrenal screening work over the years?
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From the early school years
A yearly blood or urine test for metanephrines begins, with a blood pressure check at every visit. It carries on for life.
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Preparing for the test
For the blood test you usually lie down quietly for a while before the sample is taken. Avoid coffee and tea beforehand, and bring a list of every medicine you take. Ask the laboratory for its instructions a few days ahead.
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If the result is raised
A borderline result is often repeated first. A clearly raised result leads to an MRI or CT scan to find the tumour, and sometimes a special PET scan to look for others.
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Before any operation
Blood pressure tablets are started, usually for a week or two, to block the hormones. This makes surgery far safer.
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The operation and after
Keyhole surgery removes the tumour, often keeping part of the adrenal gland. Screening then continues, because a new tumour can form on either side.
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On your report
The words you will meet, in plain language
- Phaeochromocytoma
- A tumour of the inner part of the adrenal gland that makes stress hormones. It is spelled pheochromocytoma on American reports.
- Paraganglioma
- A similar tumour that grows outside the adrenal gland, along nerves in the abdomen, chest or neck.
- Metanephrines
- The breakdown products of adrenaline and noradrenaline. They are the screening test, measured in blood or in urine.
- Catecholamines
- The family name for adrenaline, noradrenaline and similar hormones.
- Adrenal-sparing surgery
- An operation that removes the tumour but keeps part of the gland, so it can go on making the hormones you need.
- Alpha blocker
- A blood pressure tablet that blocks the effect of the stress hormones. It is given before surgery to keep blood pressure steady.
Commonly believed
Four things people assume, and what is actually true
"My blood pressure is normal, so I cannot have one."
Many VHL phaeochromocytomas are found in people with normal blood pressure. Some release hormones only now and then, and some barely at all. The blood or urine test is far more reliable than a blood pressure reading.
"I only need the test if I have symptoms."
The point of screening is to find the tumour before any symptom, because the first sign can be a dangerous crisis during surgery or childbirth. The yearly test is for everyone with VHL.
"Taking out the whole adrenal gland is the safest option."
In VHL, tumours can appear on both sides over a lifetime. If both glands are fully removed, you need steroid tablets for life. Keeping part of the gland avoids that for many people, at the cost of a small chance of regrowth.
"Only adults get these tumours."
Children with VHL can develop a phaeochromocytoma too, which is why the yearly test starts in the early school years and blood pressure is checked at every childhood visit.
Being straight with you
What this page cannot tell you
It cannot tell you whether your own result is raised enough to matter. Laboratories use different methods and different normal ranges, and results are read alongside your medicines, how the sample was taken and earlier results. An endocrinologist or the doctor who runs your VHL checks should read it. What your specific variant means is a question for the counsellor who ordered the test.
It cannot plan your surgery
Whether part of the gland can be kept, and how long to take blood pressure tablets first, depends on the tumour and on you. These are decisions for an experienced endocrine surgeon working with an endocrinologist and an anaesthetist. Ask how often the team operates on adrenal tumours, and whether keeping part of the gland is possible for you.
Who this does not apply to
Most people with high blood pressure do not have a phaeochromocytoma, and most people with headaches and sweating do not either. This page is for confirmed VHL carriers and relatives at risk who have not been tested. If you tested negative for your family's known VHL fault, you need no VHL adrenal screening.
Questions we are asked
Common questions about adrenal screening in VHL
At what age does adrenal screening start in VHL?
Usually in the early school years, with a yearly blood or urine test and a blood pressure check at every visit. It carries on for life. Families with a history of phaeochromocytoma may be advised to start earlier. Your VHL team or counsellor will set the age for your child.
Is a blood test or a urine test better?
Both measure metanephrines and both are accepted for screening. The blood test is generally slightly more sensitive and simpler for children. The urine test needs a full day's collection in a container from the laboratory. Use whichever your team recommends, and keep to the same one where you can.
Can coffee or medicines upset the result?
Yes. Coffee, tea, smoking, stress, a recent illness and some medicines, including certain antidepressants and decongestants, can raise the reading. Do not stop any medicine on your own. Give the laboratory and your doctor a full list, and follow the preparation instructions you are given.
My result is borderline. Do I have a tumour?
Not necessarily. Borderline results are common and often come from how the sample was taken or from a medicine. The usual next step is a repeat test under careful conditions. A clearly raised result, or a rising trend over several tests, is what leads to a scan. Try not to worry until the repeat is back.
Why is the test so important before surgery?
Anaesthesia and handling of the tumour can release a flood of stress hormones. In someone with an undiscovered phaeochromocytoma, that can cause a dangerous rise in blood pressure on the operating table. A recent negative test, or treatment first, makes any other operation much safer.
I have VHL and I am pregnant. What should I do?
Tell your obstetrician about VHL straight away and ask for the adrenal test if it has not been done recently. A hidden tumour can cause serious problems in pregnancy and labour. Ideally, the test is done before a planned pregnancy, so any tumour is dealt with first.
Will I need steroid tablets after surgery?
Usually not if one gland is removed, because the other keeps working. If both glands are affected, surgeons try to keep part of at least one, which avoids lifelong steroid tablets for many people. Your surgeon will explain what is possible, and what it means for you, before the operation.
Can other doctors' medicines trigger an attack?
Some can, in a person with an untreated tumour. Certain anti-sickness medicines, steroids and some blood pressure tablets started on their own are known triggers. Tell every doctor who prescribes for you that you have VHL, including your dentist, and keep a note of it in your phone and your wallet.
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) — Von Hippel-Lindau Syndrome
- National Cancer Institute — Pheochromocytoma and Paraganglioma Treatment (PDQ)
- MedlinePlus — Pheochromocytoma
- MedlinePlus Genetics — 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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Tell us what is planned and when, and bring your last metanephrine result if you have one. We will help you arrange the test and an endocrine opinion in time. One helpline serves every CION centre.