Phaeochromocytoma
What causes phaeochromocytomas?
Mostphaeochromocytomas occur for no obvious reason and without a family history of the condition particularly those affecting adults.
However, around one in every three or four occurs as part of an inherited genetic disorder, such as:
- multiple endocrine neoplasia (MEN)
- von Hippel-Lindau (VHL) syndrome
- neurofibromatosis type 1 (NF1)
These conditions cause many tumours or growths todevelop around the body for example, people with multiple endocrine neoplasia often have tumours in their thyroid gland and parathyroid glands (tiny glands near the thyroid that regulate calcium levels).
These conditions can be passed on to a child by their parents. They are passed on in what is known as an autosomal dominant pattern, which means only one parent needs to carry a gene responsible for one of these conditions to risk passing it on to their children.
There are also other rare genetic causes of phaeochromocytoma. If you are diagnosed with a phaeochromocytoma, your doctor may recommend having genetic screening for some of these conditions.
Try to describe what you experience in as much detail as you can. Keeping a diary of your symptoms may be helpful.
Your GP may want to rule out other possible causes of your symptoms such as medication or panic attacks . Phaeochromocytoma is rare compared to other causes of similar symptoms.
Your GP may arrange urine tests to measure your levels of normetadrenaline and metadrenaline (adrenaline and noradrenaline breakdown products), or may refer you to a specialist to organise blood or urine tests.
If your hormone levels are significantly higher than normal, you will be referred to a specialist doctor who may arrange a computerised tomography (CT) scan or magnetic resonance imaging (MRI) scan of your adrenal glands, or other tests.
If the scan shows a tumour on your adrenal glands, you'll probably be referred for surgery to have this removed. Its important not to do this surgery immediately, however, as some medication is required for a period of a few weeks to prepare the body for this operation.
Medically Reviewed by a doctor on 21 Dec 2018
Introduction
A phaeochromocytoma is a rare tumour of the adrenal glands, which sit above the kidneys. They are usually benign (non-cancerous), although some are cancerous.
How the adrenal glands are affected
The adrenal glands produce a range of hormones that are vital for the normal working of the body. Most phaeochromocytomas develop at the centre of one or both of the adrenal glands, in a part called
Signs andsymptoms
The symptoms of a phaeochromocytoma tend to be unpredictable often occurring in sudden attacks lasting from a few minutes to an hour. These attacks may become more frequent, longer and more severe as
What causes phaeochromocytomas?
Mostphaeochromocytomas occur for no obvious reason and without a family history of the condition particularly those affecting adults. However, around one in every three or four occurs as part of an i
Treatinga phaeochromocytoma
Most people with a phaeochromocytoma will have it surgically removed. You'll usually be given medication called alpha blockers for several weeks before the operation to block the effects of the exces
Outlook
Left untreated,phaeochromocytomas can cause severe problems and have a significant impact on your quality of life. Theeffects on your heart and blood pressure could lead to complications, some of whi
Subscribe
Ask the doctor
Rate this article
Find products