Phaeochromocytoma is a rare (usually non-cancerous) tumour of the adrenal glands, which sit above the kidneys.
The tumour can develop at any age, but is usually noticed in early to mid-adulthood.
How it affects the adrenal glands
The adrenal glands produce a range of hormones that are vital for the normal working of the body.
In most cases of phaeochromocytoma, the tumour develops at the centre of one or both of the adrenal glands, in the adrenal medulla. There may be a single tumour, or several.
The cells of the adrenal medulla make the 'fight or flight' hormones adrenaline and noradrenaline, and release these into the bloodstream when they are needed. These hormones control heart rate, metabolism and blood pressure.
The tumour causes the adrenal medulla to produce too many of these hormones, which often results in heart palpitations and high blood pressure – see below.
What are the symptoms?
The symptoms of phaeochromocytoma tend to be unpredictable – you may suddenly get an attack lasting 15-20 minutes. The three typical symptoms are:
- severe headaches
- heart palpitations
Other possible symptoms are:
- high blood pressure
- feeling irritable, nervous or anxious
- having problems sleeping
- stomach pains
- weight loss
The attacks may become more frequent, longer and more severe as the tumour grows.
However, some people with phaeochromocytoma never develop symptoms, and their condition is only discovered during tests for another problem.
What are the causes?
Phaeochromocytoma can sometimes be a part of an inherited genetic disorder, such as:
- multiple endocrine neoplasia (MEN)
- von Hippel-Lindau syndrome
The links will take you to more information on these diseases. Generally, they cause many other tumours or growths to form around the body – for example, people with MEN often have tumours in their thyroid gland and parathyroid glands (tiny glands near the thyroid that regulate calcium levels).
However, for some people with phaeochromocytoma, there is no family history and it develops spontaneously.
How is it diagnosed?
As symptoms are unpredictable, it may be hard to capture the attacks when you see your doctor – for example, your blood pressure and heart rate may be normal during the consultation.
A diagnosis of phaeochromocytoma is made when all other possible causes of your 'fight or flight' response have been ruled out – for example, your symptoms are not caused by withdrawal from drugs or panic attacks.
Your doctor will do a number of blood and urine tests to measure your levels of normetadrenaline and metadrenaline (adrenaline and noradrenaline breakdown products). Read about these tests on Lab Tests Online. If your hormone levels are significantly higher than normal, you will be referred for a CT scan or MRI scan of your adrenal glands.
If the scan shows up a mass (tumour) on your adrenal glands, you'll probably be referred for surgery to have this removed.
How is it treated?
Most people with phaeochromocytoma will have their tumour surgically removed.
You'll be given medication called alpha blockers before surgery, these will stabilise your blood pressure and pulse.
Your doctor will explain the procedure to you in detail, including the risks, and will answer any questions you have.
Read about having an operation.
After surgery, you may need to stay in intensive care to have your blood pressure and heart rate montitored.
In the rare instances where your phaeochromocytoma is cancerous, you may need chemotherapy or radiotherapy after surgery.
If your tumour cannot be removed, you will need medication to manage your condition – usually a combination of medicines to control the effects of the excessive hormones.
Most people have non-cancerous tumours that are successfully treated with surgery.
However, the tumour may return in about 1 in 10 people who have it removed, so you'll need to attend regular follow-up apointments after surgery. Call your doctor straight away if your symptoms return after having treatment for phaeochromocytoma.
High blood pressure may not be cured in some patients after surgery, although this can be managed with medication.