Phaeochromocytoma

What is a phaeochromocytoma?

Phaeochromocytoma is the medical name given to a tumour of the adrenal glands, which are located above your kidneys. It most commonly affects adults, however, children can develop it too. Most of the time, a phaeochromocytoma is not cancerous but in rare cases - around 1 in 10 - it can be malignant.

What are the symptoms of a phaeochromocytoma?

When a phaeochromocytoma develops it can cause the adrenal glands to produce too much adrenaline, resulting in an increased heart rate, heart palpitations and high blood pressure.

The symptoms are typically unpredictable, presenting as spontaneous ‘attacks’ and can last anything from a few minutes to an hour. They usually become more frequent and more noticeable as the tumour grows bigger.

The main symptoms of a phaeochromocytoma include:

Some people may never show symptoms and the condition is only discovered after undergoing tests for another condition. Symptoms can take many years to develop.

What causes a phaeochromocytoma?

There is no clear reason as to why phaeochromocytomas develop, however, they are thought to be associated with certain inherited genetic disorders such as:

  • Von Hippel-Lindau syndrome
  • Multiple endocrine neoplasia type 2
  • Neurofibromatosis type 1

If you have been diagnosed with a phaeochromocytoma, then your doctor will likely want to test to see if you also have one of these inherited disorders.

How is a phaeochromocytoma diagnosed?

As beforementioned, a phaeochromocytoma doesn’t always show symptoms and the symptoms it does present with can be quite general, so it is difficult to diagnose. It is usually picked up after undergoing scans and tests for other problems. If one is found, then you may have to undergo a further blood test, urine test, CT scan or an MRI scan.

How is phaeochromocytoma treated?

The typical approach to treatment is a type of surgery known as adrenalectomy, which will remove both the adrenal gland and the tumour. If the cancer has spread to surrounding tissues, these will also need to be removed. Before surgery, you will be given beta-blockers or alpha-blockers to block the effects of excess hormones. This will also stabilise your heart rate and blood pressure.

The surgical procedure is done under general anaesthetic and can be performed through keyhole surgery or open surgery.

  • Keyhole surgery — small incisions are made in your stomach and fine instruments are inserted and used to remove the tumour and the gland.
  • Open surgery — one large incision is made in your stomach to access the gland and tumour and remove them.

If your phaeochromocytoma was cancerous, you may need to have radiotherapy or chemotherapy following surgery.

What is recovery like?

You will have to stay in the recovery room for several hours after undergoing the surgery to ensure you recover properly from the anaesthesia. If you need something to relieve any pain, the doctor or nurse will provide you with pain killers. If you underwent keyhole surgery, you will take around 1-2 weeks to recover and 5-6 weeks if you had open surgery.

Which type of specialist treats a phaeochromocytoma?

A doctor that is specialised in diagnosing and treating a phaeochromocytoma is an endocrinologist or endocrine surgeon.

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