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.

Phaeochromocytoma

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 lymph nodes , bones or liver. 

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

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 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 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)
• headaches
• 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 phaeochromocytomas.

Diagnosing phaeochromocytomas

There are different tests to diagnose phaeochromocytomas. These include blood and urine tests, CT, MRI and PET scans, and a type of scan called an MIBG scan. Diagnosing phaeochromocytomas can be difficult. This is because they can cause a range of symptoms. And many of these can be caused by other conditions that are much more common.

Your GP will refer you to a specialist for tests if you have symptoms that could be caused by phaeochromocytoma. You might have some of the following tests.

Blood and urine tests

Doctors measure the amount of metanephrines in your urine (wee) and sometimes your blood (plasma). Metanephrines are the products made by the body when it breaks down the hormones adrenaline and noradrenaline.

Phaeochromocytomas can make large amounts of these hormones. This means the level of metanephrines in your blood and urine are higher than normal. You collect your urine over 24 hours to give a more accurate result. Your doctor will tell you how to do this. They will tell you what food and drink you should avoid for a few days before these tests. These include:

• coffee
• tea
• chocolate
• bananas
• citrus fruits

This is because they can affect your test results. Your doctor will also tell you if there are any medicines that you need to stop taking before and during the tests. For the blood test, your doctor or nurse will usually ask you to lie down for 20 to 30 minutes before they take the sample.

CT scan

A CT scanner takes x-rays from different angles to form a detailed image of the inside of your body. This can show doctors where a tumour is, how big it is and whether it has spread anywhere else. 

MRI scan

An MRI scanner uses magnetism rather than x-rays to build up a picture of the inside of your body. MRI scans are especially good at showing up soft tissue in the body.

MIBG scan

This is a specialised scan that doctors might use to diagnose phaeochromocytoma. They can also see if it has spread to other parts of your body. You have the scan in the nuclear medicine department at the hospital.

First, you have an injection of a small amount of a radioactive chemical called MIBG (meta-iodo-benzyl-guanidine). You have your scan 4 hours later. The phaeochromocytoma cells take up the MIBG, which then show up on the scan. You may have to go back to the hospital the next day to have another scan.

After you have had the radioactive injection, you need to drink plenty and go to the toilet regularly. This is to help flush out the excess MIBG and make the images on the scan clearer.

After the injection and for the rest of the day, you should avoid long periods of close contact with pregnant women and children. The team at your hospital will give you advice about this.

PET scans

DOTATATE​ PET SCAN

In some hospitals, instead of an MIBG scan, you may have a DOTATATE PET scan. This helps to diagnose phaeochromocytoma and to see if it has spread. With this type of scan you have a radioactive drug called 68-Ga-DOTATATE. This drug binds to certain receptors on tumour cells, and shows up on the scan.

You have the 68-Ga-DOTATATE as an injection into a vein in your arm. You then wait 60 to 90 minutes before having a PET scan or PET-CT scan. For the rest of the day after having the radioactive injection, you should avoid long periods of close contact with children and pregnant women. The team in the nuclear medicine department will give you advice about this.

FDG PET scan

This is the most common type of PET scan. The doctors use a different radioactive drug (tracer) called fluorodeoxyglucose (FDG). You might have this type of PET scan before your operation to remove the tumour. 

Treatment for phaeochromocytomas

The main treatment for phaeochromocytoma is surgery. Other treatments include internal radiotherapy, external radiotherapy and chemotherapy.

Your treatment depends on different factors including:

• the size of the tumour
• whether it has spread
• your general health and fitness

A team of doctors and other professionals discuss the best treatment and care for you. They are called a multidisciplinary team (MDT). The team usually includes a:

• specialist surgeon
• doctor specialising in hormone disorders (endocrinologist)
• cancer doctor (oncologist)
• doctor specialising in the diseases of tissues or cells (pathologist)
• doctor specialised in reading scans (radiologist)
• clinical nurse specialist (CNS)

You may need to travel to a hospital that specialises in phaeochromocytoma for treatment.

Treatment for phaeochromocytoma that hasn’t spread

An operation to remove the adrenal gland

Surgery is the main treatment for phaeochromocytoma. You usually have an operation to remove the adrenal gland. This is called an adrenalectomy. It will cure most people with phaeochromocytoma.

You usually have this done as keyhole surgery by a specialist surgeon. This is also called laparoscopic surgery or minimal access surgery. Your surgeon will remove the tissues surrounding the adrenal gland and the nearby lymph nodes if they think there is a risk the tumour has started to spread. They may also have to remove the kidney.

The anaesthetic and surgery can make the tumour release large amounts of adrenaline and noradrenaline. This causes very high blood pressure and can lead to serious problems. So from around 2 weeks before your operation your doctor will prescribe a medicine called phenoxybenzamine or a similar drug.

These drugs block the effect of the hormones on your blood pressure and help keep it stable. You have regular blood pressure measurements during this time. After surgery your doctor will stop the drug and your blood pressure should go back to normal after a while.

Hormone replacement

You can normally manage with the one adrenal gland if you need to have an adrenal gland removed. The remaining gland will make all the hormones you need.

You will need to have hormone replacement if you have both adrenal glands removed. You take this as tablets every day for the rest of your life.

If you can’t have surgery for phaeochromocytoma, you might have internal radiotherapy instead. We have information about internal radiotherapy below.

Check ups after treatment

You have regular check ups after your treatment. You usually have urine tests to check your metanephrine levels. You might also have further MIBG or DOTATATE scans.

You will have check ups for the rest of your life. Unfortunately in a small number of people, phaeochromocytoma can come back and spread many years later (cancerous or malignant phaeochromocytoma).

It is important to go to your follow up appointments so that if it does come back the cancer is picked up sooner.

Treatment for phaeochromocytoma that has come back or spread

Treatment will depend on several factors including:

• where the tumour has come back
• if the tumour is causing symptoms
• which treatment you may have had before for phaeochromocytoma
• your general health and fitness

Surgery

You might be able to have surgery if the phaeochromocytoma has come back in the nearby area.

Sometimes doctors recommend surgery to remove phaeochromocytoma that has spread to other parts of the body. They might suggest this if you have only a few small and well contained areas that can be taken out. If they can’t remove all the tumour, surgery may still help with controlling symptoms.

Internal radiotherapy

Radiotherapy means using radiation to treat cancer. Internal radiotherapy means having radiation treatment from inside the body.

This is called radio labelled treatment or targeted radionuclide therapy.

There are different types of radio labelled treatment. These include MIBG and DOTATATE treatments.

MIBG treatment

For this treatment, the chemical MIBG is attached to a radioactive form of iodine called iodine-131 (I-131). You have this as an injection into a vein in your arm. The MIBG circulates throughout your body in your bloodstream. Only cancer cells that produce adrenaline and noradrenaline will pick up the chemical, wherever they are in your body. The strong radiation attached to the drug then kills them.

Not everyone with phaeochromocytoma will have cells that pick up MIBG. So this treatment is not suitable for everyone. To see if this treatment might work for you, you have an MIBG scan (if you didn’t have one at diagnosis). Cells that take up MIBG will show up on your scan.

During treatment

You stay in hospital to have the MIBG treatment. This is to protect other people from the high doses of radioactivity during and immediately after your treatment. You stay in a single room, with your own bathroom, usually for about 4 to 7 days.

You are able to have visitors but they might have to talk to you from the door of your room. There will be limits on the amount of time visitors can spend with you. There will be a few restrictions for when you first get home too. You should make sure you don’t spend any more time than is absolutely necessary with young children or pregnant women. This could be for up to a week or two. Your nurse will go through all the restrictions with you before you leave.

You generally have MIBG treatment 3 times, about 12 weeks apart.

Side effects

The treatment can make you feel sick. So your nurse will give you anti sickness medicines before the treatment and for a day or two afterwards. You need to drink plenty after your treatment to help flush out the radiation.

Because your thyroid gland normally picks up iodine, you take protective iodine medicines before you begin MIBG treatment. This loads up your thyroid with normal iodine so it doesn’t pick up too much of the radioactive type. But your thyroid may pick up some radiation. So after your treatment, your thyroid might not work as well as it used to. You will have a drug called levothyroxine (a thyroid replacement hormone) if necessary.

DOTATATE treatment

DOTATATE is another chemical that is taken up by many phaeochromocytomas. The DOTATATE is joined to radioactive Lutetium or Yttrium.

This is a newer type of treatment for phaeochromocytomas. So you usually only have it if you can’t have MIBG. It is not available in all hospitals.

You have it in a similar way to MIBG treatment. Before the treatment, you have a DOTATATE scan to make sure the phaeochromocytoma takes up (absorbs) the drug.

During and after DOTATATE treatment

You have the radioactive DOTATATE as an infusion through a vein in your arm. The drug goes around your body in your bloodstream and is taken up by the cancer cells. At the same time as the infusion, you have other fluids to help protect your kidneys from the radiation.

You may have to stay in hospital to have the treatment. You will be in a single room with your own bathroom. Most people are able to go back home after a couple of days, but this depends on how quickly the radiation leaves your body.

The treatment can make you feel sick for the first few days and so your nurse will give you anti sickness medicines.

When you go home, you will need to avoid close contact with other people, particularly children and pregnant women. This can be for up to a week or two. Your team at the hospital will give you advice about this.

External radiotherapy

External radiotherapy uses specialised radiotherapy machines to aim radiation beams at a cancer from outside the body.

External beam radiotherapy can help with phaeochromocytoma that has spread. This type of treatment is helpful if the cancer has spread to the bones. It can kill the cells in the bone, reduce pain, and lower the risk of a break (fracture).

Cancer drugs

Chemotherapy

Chemotherapy uses anti cancer (cytotoxic) drugs to destroy cancer cells. You might have just one drug or a combination of more than one drug. A common combination of drugs doctors might use is cyclophosphamide, vincristine and dacarbazine (CVD).

Chemotherapy is not usually very successful in treating adrenal gland cancers. You are only likely to have this treatment if you have phaeochromocytoma that has spread to other parts of your body and has not responded to MIBG or DOTATATE treatment.

Targeted cancer drugs

Targeted cancer drugs work by ‘targeting’ the differences in cancer cells that help them to survive and grow. Researchers have looked at targeted drugs such as sunitinib for people with phaeochromocytoma that has spread. We still don’t know how well these drugs work compared to chemotherapy. 

Watch and wait

If you have phaeochromocytoma that has spread and it isn’t causing symptoms, your doctor may talk to you about delaying treatment. You have regular checks instead for a time. They may call this ‘watch and wait’ or active surveillance.

Some phaeochromocytomas grow very slowly and may not cause problems for quite some time. Cancer treatments have side effects, and so your doctor may feel these outweigh the benefit of having treatment at that time. They may suggest waiting until you get symptoms before starting treatment.

Other treatments for phaeochromocytoma that has spread

Depending on where the tumour has spread to, you may have local treatments such as:

• radiofrequency ablation (RFA)
• chemoembolisation

Coping

Finding out you have a rare tumour can be difficult to cope with. Although most phaeochromocytomas can be removed successfully, it can be hard to manage the uncertainty of not knowing whether it will come back in the future.

Your hospital team, including your specialist nurse will help support you. Being well informed about phaeochromocytoma and its treatment can make it easier to make decisions and cope with what happens.

Talking to other people who have the same type of tumour can also help.

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