This page tells you about tumours of the inner part of the adrenal gland (called phaeochromocytoma). There is information about
There are 2 adrenal glands, one above each kidney. Adrenal means next to the kidney. The adrenal glands are small, but very important because they make several hormones that help the body work properly and are vital to life. They have an inner and outer layer. The outer layer is the cortex and the inner is the medulla. Tumours can start in either of these areas. This page is about adrenal tumours that start in the medulla. They are called phaeochromocytoma (fey-oh-cromo-sy-toma). Tumours that start in the cortex are called adrenal cortical tumours.
Cancers that start in the adrenal glands are called primary adrenal gland cancers. Sometimes cancers that start in other parts of the body, such as the kidney, can spread to the adrenal glands. Doctors call this a secondary cancer. Secondary cancers respond to the same treatment as the primary. So a cancer that has spread from the kidney will be treated as a kidney cancer and not an adrenal gland cancer. If you are looking for information about a cancer that has spread to your adrenal gland, this is not the right page for you. You need to look at the treatment section for your type of primary cancer.
Phaeochromocytomas are tumours that start in the inner section of the adrenal gland (the medulla). They are a type of neuro endocrine tumour. They can be non cancerous (benign) or cancerous (malignant). Most phaeochromocytomas are benign. Only about 1 in 10 (10%) are cancerous.
Phaeochromocytomas are very rare. There is only 1 diagnosed for every 100,000 people in the UK each year. Phaeochromocytomas can happen at any age, but are more common between the ages of 30 and 60.
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), von Hippel Lindau (VHL), neurofibromatosis 1 (NF1), and familial paraganglioma syndromes (which are mostly caused by changes in the succinate dehydrogenase (SDH) gene).
Phaeochromocytomas diagnosed in children and young adults are more likely to be linked to an inherited gene change.
If you have a family history of phaeochromocytoma, or one of the family cancer syndromes, you can speak to your GP about referral to a family cancer clinic for genetic testing. If you have already been diagnosed with phaeochromocytoma, you are likely to have genetic testing to see if you have a hereditary gene change. If so, this means members of your family can then be tested for the same gene mutation. People at increased risk for phaeochromocytoma may have screening tests to pick up tumours at an early stage.
The inner part of the adrenal gland makes hormones important for the flight and fight reaction, which kicks in when we are threatened or put under sudden stress. These hormones are adrenaline (epinephrine) and noradrenaline (norepinephrine).
If you have a phaeochromocytoma, you may make too much of these hormones. An increase of adrenaline and noradrenaline can cause the following symptoms
- Anxiety or panic attacks
- Heavy sweating
- Looking very pale (pallor)
- Feeling your heart thumping in your chest (palpitations)
- Feeling or being sick
- High blood pressure
These symptoms may come on in specific attacks. Mostly they last less than 15 minutes, but they can last up to an hour. Afterwards, you usually feel very weak. The attacks become more frequent as time goes by.
Diagnosing phaeochromocytoma can be difficult as the symptoms are vague and can be caused by many other more common conditions. If you have symptoms that could be caused by phaeochromocytoma, your GP will refer you to a specialist for tests. These tests may include
Blood and urine tests
Doctors measure the amount of metanephrines in your urine and blood. 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, which means the level of metanephrines in your blood and urine are higher than normal. The urine test is usually collected over 24 hours to give a more accurate result.
Before these tests, your doctor will advise you of food and drink that you should avoid for a few days. These include coffee, tea, chocolate, bananas and 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.
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. We have detailed information about having a CT 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. We have more information about having an MRI scan.
This is a specialised scan that doctors may use to diagnose phaeochromocytoma and to 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 mildly 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 have to go back to the hospital the next day to have another scan.
After you've 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 after your scan, you should avoid long periods of close contact with pregnant women and children. The team at your hospital will give you specific advice about this.
DOTATATE PET scan
In some hospitals, instead of an MIBG scan, you may have a DOTATATE PET scan to help 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.
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.
Treatment depends on several factors, including the size of your tumour, whether it has spread and your general health and fitness. The main treatments include
Surgery to remove the affected adrenal gland will cure most benign or early stage malignant phaeochromocytomas. This operation is called an adrenalectomy. You usually have this done as keyhole surgery. This is also called laparoscopic surgery or minimal access surgery. If your surgeon thinks there is a risk that the cancer has spread into tissues close by, they will remove the tissues immediately surrounding the adrenal gland and also the nearby lymph nodes. 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. After surgery your doctor will stop the drug and your blood pressure should go back to normal after a while.
Sometimes doctors suggest surgery to remove phaeochromocytoma that has spread. They may suggest this if there are only a few small and well contained areas that can be taken out.
If you have an adrenal gland removed, you will be perfectly well with only one adrenal gland left. It will make all the hormones you need. If you have both adrenal glands removed, you will need to take hormone replacement tablets every day for the rest of your life.
After your operation, your doctor may suggest an MIBG scan or DOTATATE PET scan. These scans can show whether you need further treatment after surgery.
If you cannot have surgery, or your phaeochromocytoma has spread, you may have a type of internal radiotherapy. This is called radio labelled treatment or targeted radionuclide therapy. There are different type of radio labelled treatment. These include MIBG and DOTATATE treatments.
For this treatment, the chemical MIBG is attached to a radioactive form of iodine called iodine-131 (I-131). It is injected 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 may work for you, you will have an MIBG scan (if you didn't have one at diagnosis). If you have cells that take up MIBG this will show up on your scan.
If your doctor thinks that MIBG radiotherapy will help you, you will need to stay in hospital to have the treatment. This is to protect other people from the high doses of radioactivity during and immediately after your treatment. You will have to stay in a single room, with your own bathroom. This is usually for about 4 to 7 days. You will be able to have visitors, but they may 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 may be for up to a week or two. Your nurse will go through all the restrictions with you before you go home.
The treatment may make you feel sick and 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 need to 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 may not work as well as it used to. You will have a drug called levothyroxine (a thyroid replacement hormone) if necessary.
You may have MIBG treatment 3 times, about 12 weeks apart.
DOTATATE is another chemical that is taken up by many phaeochromocytomas. The DOTATATE is joined to radioactive Lutetium or Yttrium. This is a new type of treatment for phaeochromocytomas. It is not available in all hospitals. You have it in a similar way to MIBG treatment. Before the treatment, you will have a DOTATATE scan to make sure the phaeochromocytoma takes up (absorbs) the drug.
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. The treatment can make you feel sick for the first few days and so your nurse will give you anti sickness medicines.
You 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. When you go home, you will need to avoid close contact with other people, particularly children and pregnant women. This may be for up to a week or two. Your team at the hospital will give you specific advice about this.
Chemotherapy uses anti cancer (cytotoxic) drugs to destroy cancer cells. You may have just one drug or a combination of more than one drug. A common combination of drugs doctors may use is cyclophosphamide, vincristine and dacarbazine.
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.
We have general information about having chemotherapy.
External beam radiotherapy can help with phaeochromocytoma that has spread. The radiation beams are aimed at the treatment area from a large machine. 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 fracture. We have a section about radiotherapy treatment.
Doctors are always looking to find better ways to diagnose phaeochromocytoma, and to further improve treatments. Researchers around the world are looking at different treatments including radio labelled treatments, external radiotherapy, chemotherapy and biological therapies.
In the UK, the VIBRaNT trial is looking at a type of biological therapy called vandetanib alongside MIBG treatment. Vandetanib is a cancer growth blocker. It stops signals that cancer cells use to divide and grow. This is an early trial. The researchers are aiming to find out the best dose of vandetanib to give with MIBG treatment. The people taking part have phaeochromocytoma that can't be completely removed with surgery.
For more information about trials, you can go to our clinical trials database.
After your treatment, you will have regular check ups. If you have no sign of phaeochromocytoma on your MIBG or DOTATATE scan, it means you don't have any cells making adrenaline and noradrenaline. But non secreting cells can lie inactive or dormant for months or years before they start to make hormones again. Your doctor may repeat these scans from time to time. Mostly though, the doctor will rely on urine tests to check your metanephrine levels.
You will have check ups for this for the rest of your life. Unfortunately in a small number of people, malignant phaeochromocytoma can come back many years later. So it is important to go to your follow up appointments, so that if the cancer does come back it is picked up sooner and you can have treatment.
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