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Phaeochromocytoma

This page tells you about tumours of the inner part of the adrenal gland (called phaeochromocytoma). There is information about

 

The adrenal glands

There are two 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 medullary adrenal tumours, which are called phaeochromocytoma (fey-oh-cromo-sy-toma). Americans spell it without the a – pheochromocytoma.

If you are looking for information about adrenal cortical carcinoma (cancer starting in the outer layer of the adrenal gland) this is not the right page for you. There is information about adrenal cortical carcinoma in the question and answer section of CancerHelp UK.

 

What phaeochromocytoma is

Phaeochromocytomas are tumours that start in the inner section of the adrenal gland (the medulla). They can be non cancerous (benign) or cancerous (malignant). Most phaeochromocytomas are benign. Only about 1 in 10 (10%) are cancerous. This type of cancer is very rare and there is only 1 diagnosed for every 100,000 people in the UK each year.

Primary adrenal gland cancers start in the adrenal gland. Sometimes cancers that started 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 section for you. You need to look at the treatment section for your type of primary cancer.

 

Symptoms of phaeochromocytoma

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 adrenalin (epinephrine) and noradrenalin (norepinephrine).

If you have a phaeochromocytoma, you may make too much of these hormones. An increase of adrenalin and noradrenalin can cause the following symptoms

  • Anxiety or panic attacks
  • Heavy sweating
  • Headaches
  • 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.

 

Treating phaeochromocytomas

Your doctor may suggest

Surgery

Surgery to remove the affected adrenal gland will cure most benign or early stage malignant phaeochromocytomas. This operation is called an adrenalectomy. 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.

The anaesthetic and surgery can make the tumour release large amounts of adrenalin and noradrenalin. This causes very high blood pressure and can lead to serious problems. So for up to 10 days before your operation your doctor will prescribe a medicine called phenoxybenzamine or a similar drug. These drugs block the tumour from making these hormones. After surgery your blood pressure should go back to normal after a while.

Sometimes doctors suggest surgery to remove phaeochromocytoma that has spread. They only suggest this if there are only one or two small and well contained areas.

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.

After your operation, your doctor may suggest an MIBG scan (see below). This type of scan picks up any cells that make adrenalin or noradrenalin wherever they are in the body. It can show whether you need further treatment after surgery.

MIBG radiotherapy

If the phaeochromocytoma has spread, your doctor may offer radiotherapy, using a treatment called MIBG (Meta-ldo-Benzyl-Guanidine). MIBG is a chemical readily picked up by many phaeochromocytomas. For this treatment, the MIBG is attached to a radioactive form of iodine called radioiodine or 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 adrenalin and noradrenalin will pick up the chemical, wherever they are in your body. The radiation attached to the iodine 131 then kills them.

Not everyone with phaeochromocytoma has cells that make too much adrenalin and noradrenalin. 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 have cells that make adrenalin and noradrenalin, the scan will show an uptake of MIBG.

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, for about 7 to 10 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 the first few days at home too. You should make sure you don't spend any more time than is absolutely necessary with young children or pregnant women. Your nurse will go through all the restrictions with you before you go home.

Because your thyroid gland normally picks up iodine, you need to take 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, it may not work as well as it used to. You will have a drug called levothyroxine (a thyroid replacement hormone) if necessary.

MIBG is still an experimental treatment for phaeochromocytomas. We don't yet know how well it works in the long term.

Chemotherapy

Chemotherapy uses anti cancer (cytotoxic) drugs to destroy cancer cells. You may have just one drug or a combination of more than one drug. 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 not responded to MIBG and has spread to other parts of the body. There is detailed information about chemotherapy in CancerHelp UK.

Radiotherapy

External beam radiotherapy can help with MIBG that has spread. This is the usual type of radiotherapy treatment. 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. There is a section about radiotherapy treatment in CancerHelp UK.

 

Check ups after treatment

After your treatment, you will have regular check ups. If you've had no sign of any phaeochromocytoma on your MIBG scan, it means you don't have any cells making adrenalin and noradrenalin. But the cells can begin to make these hormones in the future. Non secreting cells can lie dormant for months or years. Your doctor may repeat MIBG scans from time to time and treat you again if there is uptake. Mostly though, the doctor will rely on urine tests to check your adrenalin and noradrenalin levels.

You will have check ups for this for the rest of your life. Unfortunately in a small number of people, phaeochromocytoma cancer can come back many years later. But in about 9 out of 10 people (90%) the cancer won't come back.

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Updated: 21 September 2011