What are phaeochromocytomas?

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

Types of adrenal gland tumours

Tumours of the adrenal glands can start in any part of the adrenal gland. There are 3 types of adrenal gland tumours:

• adrenal cortical cancer (ACC)

• phaeochromocytoma (pronounced fey-oh-cromo-sy-toma)

• adenomas - these are not cancer, they are ​benign​    

This information is about phaeochromocytoma. Phaeochromocytomas are adrenal tumours that start in the medulla.

We have separate information about adrenal cortical tumours (ACCs). These tumours start in the cortex.

Go to information about adrenal cortical 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. 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.

Go to the A to Z list of cancer types

Phaeochromocytomas and paragangliomas

Phaeochromocytoma

Most phaeochromocytomas do not spread to other areas of the body. Only about 10 in 100 phaeochromocytomas (about 10%) spread to parts of the body such as the ​lymph nodes, bones or liver. Metastatic phaeochromocytomas are phaeochromocytomas that spread to other parts of the body.

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.

Paragangliomas

Paragangliomas are similar tumours to phaeochromocytomas, but they form outside the adrenal glands. They are even rarer. They start in the same type of neuroendocrine cells as phaeochromocytomas. Paragangliomas develop in the head, neck, chest, tummy (abdomen) or ​pelvis.

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 depends on if they release hormones or not. You might hear doctors use the terms non functioning and functioning:

• Non functioning tumours make and release normal levels of hormones.

• Functioning tumours make and release abnormal levels of hormones.

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 35 in 100 cases of phaeochromocytoma (about 35%) 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)​

Phaeochromocytoma can happen at any age, but the average age of being diagnosed is between 44 and 47. 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)

• headaches

• sweating

• high blood pressure

• anxiety or panic attacks

• looking very pale

• feeling weak

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.

Find out about the tests you might have to diagnose phaeochromocytomas