Phaeochromocytoma (pheochromocytoma): symptoms, tests and treatment

The NHS says the symptoms tend to be unpredictable and often occur 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. Cancer Research UK says mostly they last less than 15 minutes but can last up to an hour, that you usually feel very weak afterwards, and that attacks become more frequent as time goes by.

The symptoms come from the tumour making too much adrenaline and noradrenaline, the hormones behind the body's fight or flight reaction. Because the symptoms resemble many more common problems, including anxiety and panic attacks, the diagnosis is easy to miss.

The NHS says some people have symptoms for many years before a phaeochromocytoma is diagnosed, while others have no symptoms at all.

• Headaches

• Heavy sweating

• Heart palpitations (a thumping heartbeat)

• High blood pressure

• A pale face

• Feeling or being sick

• Feeling anxious or panicky

• Shakiness (tremor) and feeling weak

The NHS says diagnosis can be difficult because the symptoms are quite general and could be caused by many other more common conditions. An adrenal gland tumour is sometimes found on a scan done for another reason.

Cancer Research UK says your doctor will ask for a metanephrine test. Metanephrines are made when the body breaks down adrenaline and noradrenaline, and phaeochromocytomas can raise their level in urine and sometimes in blood (plasma). A 24-hour urine collection gives a more accurate result. You will be told what to avoid beforehand, which includes coffee, tea, cola, nicotine or smoke, paracetamol and exercise, and which medicines to stop. For the blood test you usually lie down for 30 minutes before the sample is taken.

Scans then show where the tumour is, how big it is and whether it has spread. If the tumour has been found, genetic testing may be recommended, and Cancer Research UK says doctors may test family members for the same gene change if you have an inherited one.

The NHS says most people will need surgery to remove it, and that you will usually be given alpha blockers (and in some cases beta blockers) for several weeks before the operation to block the effects of the excess hormones and stabilise heart rate and blood pressure. Cancer Research UK says that from around 2 weeks before the operation your doctor will prescribe phenoxybenzamine or a similar drug, because anaesthetic and surgery can make the tumour release large amounts of hormones and cause very high blood pressure.

The operation is carried out under general anaesthetic. The NHS says it may be keyhole (laparoscopic) surgery, the most common type, or open surgery with a single larger incision, usually in the tummy. Cancer Research UK says surgery usually removes the adrenal gland (adrenalectomy) and will cure most people. You can usually manage with one adrenal gland; hormone replacement tablets are needed for life if both glands are removed.

If the tumour is cancerous, the NHS says chemotherapy or radiotherapy may be needed in addition to surgery, and if it cannot be removed, medicines to control the effects of excess hormones are used. Cancer Research UK also describes radioisotope therapy such as MIBG treatment for tumours that have come back or spread, and says a multidisciplinary team, which may include a surgeon, an endocrinologist and an oncologist, decides your plan. You may need to travel to a hospital that specialises in phaeochromocytoma.

Cancer Research UK explains that phaeochromocytomas are rare neuroendocrine tumours that start in the inner part of the adrenal gland, called the medulla, which makes the hormones adrenaline and noradrenaline. There are two adrenal glands, one above each kidney. The NHS says the tumour is mainly found in adults, although children can sometimes develop one, and Cancer Research UK gives the average age at diagnosis as between 44 and 47.

Paragangliomas are similar tumours that form outside the adrenal glands, in the head, neck, chest, tummy or pelvis, and are even rarer. Doctors treat them in a similar way.

The NHS says many phaeochromocytomas occur for no obvious reason and do not run in the family. However, up to 1 in 3 occurs as part of an inherited genetic disorder. Cancer Research UK says doctors think about 35 in 100 cases are linked to an inherited gene change, including rare syndromes such as multiple endocrine neoplasia type 2 (MEN 2), neurofibromatosis 1 and von Hippel-Lindau syndrome, and that tumours in children and young adults are more likely to be linked to an inherited change.

Cancer Research UK advises speaking to your GP if you have a family history of phaeochromocytoma or one of these syndromes, as they can refer you to a family cancer clinic for assessment.

The NHS says that without treatment phaeochromocytomas can cause severe problems, with a 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. The NHS says you will need regular check-ups after surgery so that it can be picked up and treated as soon as possible, and to contact your GP immediately if symptoms return. Cancer Research UK says check-ups usually include urine tests of metanephrine levels and sometimes MIBG or DOTATATE scans, and continue for the rest of your life.

The sources read for this guide do not describe a specific emergency threshold for a suspected phaeochromocytoma, so use general emergency advice. Call 999 or go to A&E for severe chest pain, a very severe sudden headache, signs of a stroke such as a drooping face, arm weakness or slurred speech, or collapse. Call NHS 111 if you are unsure.

Contact your GP promptly if you have repeated attacks of headache, sweating and palpitations, particularly with high blood pressure, or if you have been treated for a phaeochromocytoma and symptoms return.

A GP usually starts the process by checking blood pressure and arranging blood or urine tests, then refers you to an endocrinologist if a phaeochromocytoma is suspected. Treatment is planned by a multidisciplinary team and, as Cancer Research UK notes, may be at a specialist hospital.

Private specialist consultations and scans are an alternative route to the same tests, but surgery for this rare tumour is usually done at centres with specific experience, so ask where your team treats this condition and who is part of the multidisciplinary team.