CION Cancer Clinics
Screening for phaeochromocytoma before any surgery in MEN2 | CION Cancer Clinics
If you or your child carries a RET change linked to MEN2, a hidden adrenal tumour called a phaeochromocytoma must be ruled out before any operation. An unknown one can send blood pressure dangerously high under anaesthesia. The check is a simple blood or urine test. This page explains why it matters, when it is done, and what happens if the result is positive. At CION Cancer Clinics, our oncologists plan screening and care for families with an inherited cancer syndrome, explained in plain words.
On this page
- Why must a phaeochromocytoma be ruled out before surgery in MEN2?
- Which operations and situations need the check first?
- How is a phaeochromocytoma screened for before surgery?
- The words you will meet, in plain language
- What this page cannot tell you
- Four things families tell us, and what is actually true
- Common questions about adrenal screening before surgery
The short answer
Why must a phaeochromocytoma be ruled out before surgery in MEN2?
A phaeochromocytoma is an adrenal tumour that releases bursts of adrenaline-like hormones. Anaesthesia, surgery and childbirth can trigger a surge that sends blood pressure and heart rate dangerously high. If the team knows about the tumour beforehand, they prepare the body with tablets and the operation can go ahead safely.
Who needs this check
Anyone with MEN2A or MEN2B, and anyone carrying a RET change that carries adrenal risk. The check is done before thyroid surgery, before any other planned operation, and before a planned pregnancy. It applies to children with the highest-risk changes as well as adults.
Why the adrenal comes before the thyroid
If both a phaeochromocytoma and thyroid cancer are found at the same time, the adrenal tumour is removed first. Operating on the thyroid with an untreated phaeochromocytoma is exactly the situation this screening exists to prevent. The thyroid operation follows once the adrenal tumour is dealt with.
A normal result on the day protects that operation. It does not replace the regular checks that follow.Before what, exactly
Which operations and situations need the check first?
The rule is simple. If a RET carrier is about to be put under stress, the adrenal glands are checked first.
Thyroid surgery
Preventive or cancer surgery on the thyroid is the most common reason for the check. The surgeon will not usually book the operation until a recent adrenal test is on file.
Any other planned operation
A hernia repair, gall bladder removal, a dental procedure under general anaesthesia or a scope under deep sedation. What matters is the anaesthetic and the stress on the body, not the size of the operation.
Pregnancy and childbirth
An unknown phaeochromocytoma is dangerous in pregnancy, for mother and baby. Women who carry a RET change are advised to be checked before trying to conceive, and the obstetric team should know about MEN2 from the first visit.
New medicines
Some ordinary medicines can trigger a surge in a person with an untreated phaeochromocytoma.
Tell the doctor about MEN2 before
- Starting certain blood pressure tablets on their own
- Some anti-sickness medicines and steroids
- Any scan that uses an injected medicine
Not sure whether this applies to you?
Ask an oncologistSudden attacks of a pounding headache, a racing or thumping heart, heavy sweating, chest pain or a sense that something is badly wrong can mean a phaeochromocytoma is releasing hormones. If you carry a RET change and this happens, go to the nearest emergency department the same day. Tell them you have MEN2 or a RET change. Do not wait for your next check-up, and do not start a new blood pressure tablet on your own.
Step by step
How is a phaeochromocytoma screened for before surgery?
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You tell the team about MEN2
Every surgeon, anaesthetist and obstetrician needs to hear it from you. Carry a copy of the RET report and your last adrenal result.
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A blood or urine test
The test measures metanephrines, the breakdown products of adrenaline. A blood sample is simplest. Some centres use a full day's urine collection instead.
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A scan if the result is raised
A CT or MRI of the adrenal glands shows whether a tumour is present, its size and whether one or both sides are involved.
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Preparation with tablets
If a tumour is found, tablets that relax the blood vessels are given for a period before surgery. You will also be asked to drink more and add salt, which keeps blood pressure steady during the operation.
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Adrenal surgery first
The tumour is removed, usually by keyhole surgery. Where possible the surgeon keeps healthy adrenal tissue to protect your own steroid hormones.
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The planned operation follows
Once the adrenal is treated and you have recovered, the thyroid operation or other planned surgery can go ahead safely.
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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 too much adrenaline or noradrenaline. In MEN2 it is almost always benign.
- Adrenal glands
- Two small glands sitting on top of the kidneys. They make stress hormones and the steroid hormones the body needs every day.
- Metanephrines
- What adrenaline and noradrenaline break down into. A high level in the blood or urine suggests a phaeochromocytoma.
- Alpha-blocker
- A tablet that relaxes the blood vessels. It is the usual preparation before a phaeochromocytoma is removed.
- Cortical-sparing surgery
- An operation that removes the tumour but keeps the outer layer of the adrenal, so the body can still make its own steroid hormones.
- Bilateral
- On both sides. In MEN2 a phaeochromocytoma can appear in both adrenal glands, sometimes years apart.
Being straight with you
What this page cannot tell you
It cannot tell you when your own screening should start or how often it should be repeated. That depends on the exact RET change in your family, your age and your earlier results. Your endocrinologist sets the schedule, and it can differ between two members of the same family.
It cannot interpret a result you are holding
A metanephrine level can be mildly raised for reasons that have nothing to do with a tumour, including some medicines and stress on the day of the test. What your specific variant means is a question for the counsellor who ordered the test. A borderline adrenal result is a question for your endocrinologist.
Who this does not apply to
Most people having surgery do not need this test. It is for people with MEN2 or a RET change. Relatives who tested negative for the family's RET change do not need adrenal screening. MEN1 is a different condition, and its adrenal growths are usually of a different kind.
If you are not sure whether your RET result means you need this check, ask before the operation is booked, not on the day.Commonly believed
Four things families tell us, and what is actually true
Many phaeochromocytomas found in MEN2 cause no symptoms at all, because regular screening finds them early. Feeling well is not a reason to skip the test before an operation.
A phaeochromocytoma can develop after a normal result. Before a planned operation the team wants a recent test. An old one may not reflect what is happening now.
The danger comes from the anaesthetic and the stress on the body, not from the size of the operation. A dental extraction under general anaesthesia carries the same risk as a larger procedure.
Some RET changes carry little adrenal risk, but few teams will assume it without testing. Your endocrinologist will tell you whether screening applies to your family's change.
Questions we are asked
Common questions about adrenal screening before surgery
What happens if a phaeochromocytoma is missed before surgery?
The anaesthetic and the operation can trigger a sudden surge of hormones. Blood pressure and heart rate can rise to dangerous levels, putting strain on the heart and brain. This is rare when screening is done, which is the whole point of doing it first.
Do I need to fast or stop medicines before the blood test?
Some centres ask you to rest lying down before the sample is taken, and a few medicines can affect the reading. Your team will tell you what to do. Do not stop any regular medicine on your own without asking first.
How recent does the test need to be?
It should be recent enough for the surgical team to trust it, and each team sets its own rule. If your last test was some time ago, expect to repeat it before a planned operation. Ask the surgeon's office when you book.
Does my child need this before thyroid surgery?
It depends on the exact RET change. For the highest-risk changes, adrenal screening starts in later childhood. For a very young child having early thyroid surgery, the team decides whether a test is needed first. Your endocrinologist will explain which applies to your child.
If a tumour is found, will both adrenal glands be removed?
Not usually. If only one side is affected, only that side is operated on. Where both are affected, surgeons often try to keep part of one gland. This can spare you lifelong steroid tablets. The choice depends on the size and position of each tumour.
I am pregnant and carry a RET change. What should I do?
Tell your obstetrician and endocrinologist straight away. A blood test can be done safely in pregnancy. If a phaeochromocytoma is found, your team will plan treatment and delivery together to keep you and the baby safe.
Is this test needed for MEN1 too?
MEN1 can cause adrenal growths, but they are rarely phaeochromocytomas. Your endocrinologist may still check adrenal hormones if a scan shows a growth. The routine screening before surgery described here is a MEN2 rule.
Where can I get screened in Hyderabad?
The blood test is available through most large laboratories, but it should be ordered and read by an endocrinologist who knows your RET result. Call the CION helpline before your surgery is booked, and someone will point you to the right clinic.
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) — Multiple Endocrine Neoplasia Type 2
- NHS — Phaeochromocytoma
- National Cancer Institute — Pheochromocytoma and Paraganglioma Treatment (PDQ) - Patient Version
- National Cancer Institute — Genetics of Endocrine and Neuroendocrine Neoplasias (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.
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Talk to us
Have surgery coming up and carry a RET change?
Tell us what operation is planned and when your adrenals were last checked. We can help arrange the screening and make sure the surgical team has the result in time. One helpline serves every CION centre.