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Treatment for phaeochromocytomas

The main treatments for phaeochromocytomas are surgery, internal radiotherapy, external radiotherapy and chemotherapy.

Your treatment depends on:

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

Surgery

An operation to remove the adrenal gland

Surgery to remove the affected adrenal gland will cure most non cancerous (benign) or early stage malignant (cancerous) 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. Your surgeon will remove the tissues immediately surrounding the adrenal gland and the nearby lymph nodes if they think there is a risk that the cancer has spread into tissues close by. 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.

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.

Surgery to remove cancer spread

Sometimes doctors recommend surgery to remove phaeochromocytoma that has spread. They might suggest this if you have only a few small and well contained areas that can be taken out.

Further treatment

After your operation, your doctor might recommend you have an MIBG scan or DOTATATE PET scan. These scans can show whether you need further treatment after surgery.

Types of internal radiotherapy

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

You might have internal radiotherapy if you can't have surgery or your phaeochromocytoma has spread. 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 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 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 new and more experimental 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 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 specific advice about this.

External radiotherapy

External radiotherapy uses specialised radiotherapy machines to aim radiation beams at a cancer.

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 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.

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’ those differences that help a cancer cell to survive and grow. Researchers have looked at targeted drugs for people with phaeochromocytoma that has spread. We still don't know how well these drugs work compared to chemotherapy. 

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 this for the rest of your life. Unfortunately in a small number of people, malignant phaeochromocytoma can come back many years later.

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.

Last reviewed: 
22 Jun 2018
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