Phaeochromocytomas are rare cancers that start in the inner section of the adrenal gland (the medulla). The adrenal gland medulla makes the hormones adrenaline and noradrenaline. Phaeochromocytomas can make too much of these hormones. This can cause symptoms including sweating, headaches and high blood pressure.
The adrenal glands
There are 2 adrenal glands, one above each kidney. Adrenal means next to the kidney.
The adrenal glands are small, but very important. They make hormones that help the body work properly and are vital to life.
The adrenal glands have an inner and outer layer:
- cortex - outer layer
- medulla - inner layer
Tumours of the adrenal glands can start in either of these areas.
Adrenal tumours that start in the medulla are called phaeochromocytoma (fey-oh-cromo-sy-toma). Tumours that start in the cortex are called adrenal cortical tumours.
The inner part of the adrenal gland makes hormones that are important for the "flight and fight" reaction. This kicks in when we are threatened or put under sudden stress. These hormones are adrenaline (epinephrine) and noradrenaline (norepinephrine).
Cancers that spread to the adrenal glands
Cancer can start to grow elsewhere in the body and spread to the adrenal glands. This is secondary cancer. Secondary cancer in the adrenal glands is more common than cancer that starts in the adrenal glands.
For example, kidney cancer can spread to the adrenal glands. This is known as secondary cancer and would be treated as kidney cancer, not an adrenal gland cancer.
You should look for information about your primary cancer type if you cancer has spread to the adrenal glands.
What is phaeochromocytoma?
Phaeochromocytomas are tumours that start in the inner section of the adrenal gland (the medulla). They are a type of neuroendocrine tumour.
Phaeochromocytomas are very rare. There is only 1 diagnosed for every 100,000 people in the UK each year.
They can be non cancerous (benign) or cancerous (malignant). Most phaeochromocytomas are benign. Only about 10 in 100 (10%) are cancerous.
Phaeochromocytomas can happen at any age, but are more common between the ages of 30 and 60.
Causes of phaeochromocytoma
As with many types of cancer, it is not known what causes phaeochromocytoma. But doctors know that having a family history of phaeochromocytoma increases your risk of developing it. This means there is a change in a gene (mutation) that can be passed on within a family. Doctors think about 1 in 4 cases of phaeochromocytoma (25%) are linked to an inherited gene change.
Phaeochromocytoma can be part of a family cancer syndrome, where an inherited gene change causes a number of cancers to develop within a family.
These inherited syndromes are rare. They include:
- multiple endocrine neoplasia type 2 (MEN 2)
- neurofibromatosis 1 (NF1)
- familial paraganglioma syndromes, which are mostly caused by changes in the succinate dehydrogenase (SDH) gene
- von Hippel Lindau disease
Phaeochromocytomas diagnosed in children and young adults are more likely to be linked to an inherited gene change.
If you have an increased risk
Speak to your GP if you have a family history of phaeochromocytoma or one of the family cancer syndromes. They can refer you a family cancer clinic for assessment.
You are likely to have genetic testing if you are diagnosed with phaeochromocytoma. If you are found to have a hereditary gene change, members of your family might also be tested for the same gene mutation.
People at an increased risk of developing phaeochromocytoma might have screening tests to pick up tumours at an early stage.
Symptoms of phaeochromocytoma
The inner part of the adrenal gland makes hormones that are important for the flight and fight reaction. This kicks in when we are threatened or put under sudden stress. These hormones are adrenaline (epinephrine) and noradrenaline (norepinephrine).
You might make too much of these hormones if you have a phaeochromocytoma. An increase of adrenaline and noradrenaline can cause the following symptoms:
- anxiety or panic attacks
- flushing or sweating
- looking very pale (pallor)
- feeling your heart thumping in your chest (palpitations)
- feeling or being sick
- high blood pressure
These symptoms might come on in specific attacks. Mostly they last less than 15 minutes, but they can last up to an hour. You usually feel very weak afterwards. The attacks become more frequent as time goes by.