Phaeochromocytoma

What is phaeochromocytoma?

Phaeochromocytoma is usually benign and doesn't spread to other organs, although one in ten is cancerous.

Symptoms

A phaeochromocytoma develops from the cells which make adrenaline and the related chemical noradrenaline. Overproduction of adrenaline and noradrenaline causes a variety of symptoms, especially high blood pressure and weight loss.

Those affected typically describe episodes in which they get headaches, flushing and a fast or irregular heartbeat (palpitations), which may be linked to sudden increases in blood pressure. These may be triggered by exercise, emotion or simple body movement, and may occur every few weeks or several times a day, lasting just a few seconds or a number of hours.

Other symptoms of the attacks may include:

• Profuse sweating
• Anxiety
• Depression
• Nausea
• Vomiting
• Rapid breathing
• Pallor
• Numbness, tingling and/or tremor
• Abdominal pain

The attacks tend to worsen with time, occurring more frequently and becoming more severe as the tumour grows.

But some people have no symptoms at all – about ten per cent of pheochromocytomas are discovered by chance.

If untreated, phaeochromocytoma may cause stroke or damage to the heart, kidneys and brain.

Men and women can be affected. It's most common between 40 and 60, but can occur at any age.

Causes and risk factors

It isn't known what causes the tumour to develop in most cases, but it leads the adrenal gland to secrete excessive amounts of the hormones adrenaline and noradrenaline.

Phaeochromocytoma may be associated with other endocrine gland tumours.

Around ten per cent are linked to rare inherited genetic syndromes.

The diagnosis is made using a combination of blood and urine tests and imaging of the adrenal glands, for example using CT scan and MRI, to look for a tumour and any evidence that it may be malignant and has spread.

Treatment and recovery

Treatment involves drugs to bring blood pressure to normal levels, followed by surgery to remove the tumour. Blood pressure and adrenaline/noradrenaline levels must be checked for some time afterwards to ensure that removal of the tumour was complete. If the tumour was benign then survival rates are high, but in people affected by malignant pheochromocytomas, less than 50 per cent survive longer than five years.

Since the cause of phaeochromocytoma is unknown, it isn't possible to prevent it.