What are phaeochromocytomas?
Phaeochromocytomas are rare tumours that start in the inner part of the adrenal gland (the medulla). They are a type of neuroendocrine tumour. They can happen at any age but are more common between the ages of 30 and 60.
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 phaeochromocytomas (fey-oh-cromo-sy-tomas). Tumours that start in the cortex are called adrenal cortical tumours.
Phaeochromocytomas can be non cancerous (benign) or cancerous (malignant). Most phaeochromocytomas are benign and do not spread to other areas of the body. Only about 10 in 100 phaeochromocytomas (10%) are malignant and may spread to parts of the body such as the
Doctors usually can’t tell in advance which tumours will come back or spread. So everyone with a phaeochromocytoma has regular check ups after treatment.
Similar tumours to phaeochromocytomas can form outside the adrenal glands and are even more rare. These are paragangliomas. They start in the same type of neuroendocrine cells as phaeochromocytomas. Paragangliomas are found in the head, neck, chest, tummy (abdomen) or
Some paragangliomas cause the same symptoms as phaeochromocytomas. This is because they release the same types of hormones. Paragangliomas that don’t release hormones may still cause symptoms, such as pain. This depends on their size and where they are in the body.
Doctors treat paragangliomas in a similar way to phaeochromocytomas. Treatment will depend on if they release hormones (functioning tumours) or not (non functional tumours).
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. Doctors would treat this as kidney cancer, not an adrenal gland cancer.
You should look for information about your type of primary cancer if your cancer has spread to the adrenal glands.
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 25 in 100 cases of phaeochromocytoma (about 25%) are linked to an inherited gene change.
Phaeochromocytoma can be part of a family cancer syndrome. This means 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
- von Hippel-Lindau disease (VHL)
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 to a family cancer clinic for assessment.
You are likely to have genetic testing if you are diagnosed with phaeochromocytoma. If you do have a hereditary gene change, doctors may test members of your family 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 body's 'fight or flight' 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:
- feeling your heart thumping in your chest (palpitations)
- flushing or sweating
- high blood pressure
- anxiety or panic attacks
- looking very pale
- feeling or being sick
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.
Some phaeochromocytomas are picked up before they cause symptoms. This might be after having a scan for something else. Or as part of screening for an increased risk of phaeochromocytoma.