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.
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
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.
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.
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.
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.
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 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 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).
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:
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.
Our discussion forum Cancer Chat is a place for anyone affected by cancer. You can share experiences, stories and information with other people who know what you are going through.
Cancer Research UK nurses provide general information and support. You can call our nurse freephone helpline on 0808 800 4040. They are available from Monday to Friday, 9am to 5pm. Or you can send them a question online.
Other support organisations
There are also other support organisations that can help. These include:
Association for multiple endocrine neoplasia disorders (AMEND)
The Association for Multiple Endocrine Neoplasia Disorders (AMEND) is a patient group which was set up to support people affected by multiple endocrine neoplasia disorders. This includes providing information about phaeochromocytomas and paragangliomas. They also offer a free counselling service for its members and their families.
Neuroendocrine cancer UK
Neuroendocrine UK help support people with neuroendocrine tumours and their families. This includes providing information about phaeochromocytomas and paragangliomas. You can talk to one of their specialist nurses or counsellors, join one of the local support groups or be part of their online community, or go to one of their education events.