Phaeochromocytoma

How the tumour affects the adrenal glands

The adrenal glands make the "fight or flight" hormones adrenaline and noradrenaline. These hormones are released into the bloodstream when needed.

The hormones control heart rate, blood pressure and metabolism (the chemical processes that keep your organs working).

A phaeochromocytoma can cause the adrenal glands to produce too much of these hormones, which often results in problems such as heart palpitations and high blood pressure.

Symptoms of a phaeochromocytoma

The symptoms of a phaeochromocytoma tend to be unpredictable, often occurring in sudden attacks lasting from a few minutes to an hour.

The attacks may last longer, and become more frequent and severe, as the tumour grows.

The symptoms and signs of a phaeochromocytoma can include:

• headaches
• heavy sweating
• a rapid heartbeat (tachycardia)
• high blood pressure (hypertension)
• a pale face (your face may look grey)
• feeling or being sick
• feeling anxious or panicky
• shakiness (tremor)

Some people with a phaeochromocytoma don't have symptoms. Their condition is either never diagnosed or only discovered during tests for another problem.

Others have symptoms for many years before a phaeochromocytoma is diagnosed.

Causes of a phaeochromocytoma

Many phaeochromocytomas occur for no obvious reason and don't run in the family.

Up to 1 in every 3 phaeochromocytomas occurs as part of an inherited genetic disorder, such as:

• multiple endocrine neoplasia type 2 (MEN)
• von Hippel-Lindau (VHL) syndrome
• neurofibromatosis type 1 (NF1)

These genetic disorders cause different tumours or growths to develop around the body.

If you're suspected of having a phaeochromocytoma, your doctor will refer you to a specialist. A hospital specialist will, depending on your symptoms, decide if it is necessary to investigate to see if you might have these conditions.

Diagnosing a phaeochromocytoma

Diagnosing a phaeochromocytoma can be difficult. This is because the symptoms are quite general and could be caused by many other more common conditions.

An adrenal gland tumour is sometimes discovered during a scan for another reason. In this case, you'd have further tests to find out more about the tumour, which may include:

• blood and urine tests
• a CT scan
• an MRI scan

Cancer Research UK has more information about the tests and scans that are used to help diagnose a phaeochromocytoma.

Treating a phaeochromocytoma

Most people with a phaeochromocytoma will need surgery to have it removed.

You'll usually be given medication for several weeks before the operation, to block the effects of the excess hormones on your body and stabilise your heart rate and blood pressure.

Your doctor will discuss the best type of operation for you and explain the procedure in detail, including the risks. They'll also answer any questions you have.

If your phaeochromocytoma is cancerous, you may need chemotherapy or radiotherapy as well as surgery.

If your tumour can't be removed, you'll need medication to manage your condition. This will usually be a combination of medicines to control the effects of the excessive hormones.

Outlook

Left untreated, phaeochromocytomas can cause severe problems and significantly affect your quality of life.

You'll be at risk of an irregular heartbeat (arrhythmia), heart attacks, strokes and organ failure.

Most tumours can be successfully removed by surgery and this will usually mean most of the symptoms disappear.

In a small number of people, the tumour may come back. You'll therefore need regular check-ups following surgery so that, if it does return, it can be picked up and treated as soon as possible. Contact your GP immediately if your symptoms come back.

The Cancer Research UK website has more information about phaeochromocytomas.

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