This information is about a specific type of brain tumour called a haemangioblastoma. It should ideally be read with our general information about brain tumours.

Cells within the brain normally grow in an orderly and controlled way, but if this order is disrupted for some reason, the cells continue to divide and form a lump or tumour.

A tumour may be either benign or malignant. Although a benign tumour can continue to grow, the cells do not spread from the original site. In a malignant tumour, the cells can invade and destroy surrounding tissue and may spread to other parts of the brain.

Tumours affecting the central nervous system (CNS), which is made up of the brain and the spinal cord, are fairly rare. About 4500 new tumours are diagnosed each year in the UK. Many of these are malignant.

Most brain tumours are named after the type of cells from which they develop. A haemangioblastoma tumour is formed from cells that line the blood vessels, at an early stage of their development. It is almost always a benign tumour, which may be like a cyst (a fluid-filled area), and more than one tumour may be present.

Haemangioblastomas are usually found in the cerebellum, at the back of the brain. They are commonly slow-growing tumours and do not spread to other parts of the brain.

Haemangioblastomas can be found in adults and children, and mostly occur as part of a rare hereditary condition known as von Hippel-Lindau disease (VHL).

Haemangioblastomas are slow-growing tumours, and any signs and symptoms usually develop slowly over several years.

The main symptoms arise from increased pressure within the skull (raised intracranial pressure). This may be due to a blockage in the ventricles (fluid-filled spaces in the brain) that leads to a build-up of cerebrospinal fluid (CSF). CSF is the fluid that surrounds and protects the brain and spinal cord. The increased pressure may also be caused by the tumour itself.

Raised intracranial pressure can cause headaches, sickness (vomiting) and sight problems. A condition called nystagmus (jerky eye movements) is commonly seen with this type of tumour. Changes in behaviour and personality are both general signs that a brain tumour may be present.

Haemangioblastomas are usually found in the cerebellum, the area of the brain that controls coordination and balance. A tumour in this part of the brain often causes problems with walking. People may stumble, or walk awkwardly, and have problems keeping their balance. These tumours may also develop in the spinal cord. This may cause back and neck pain, numbness and weakness in the arms or legs, or both.

So that your doctors can plan your treatment, they need to find out as much as possible about the type, position and size of the tumour, so you may have a number of tests and investigations.

Neurological examinations (nerve tests) You will usually have a neurological examination to assess any effect of the tumour on your nervous system. A CT scan or MRI scan will be done to find the exact position and size of the tumour.

CT (computerised tomography) scan A CT scan takes a series of x-rays which build up a three-dimensional picture of the inside of the body. The scan is painless and takes from 10–30 minutes. CT scans use a small amount of radiation, which will be very unlikely to harm you and will not harm anyone you come into contact with. You will be asked not to eat or drink for at least four hours before the scan.

Most people who have a CT scan are given a drink or injection to allow particular areas to be seen more clearly. This may make you feel hot all over. Before having the injection or drink, it is important to tell the person doing this test if you are allergic to iodine or have asthma.

MRI (magnetic resonance imaging) scan This test is similar to a CT scan, but uses magnetism instead of x-rays to build up a detailed picture of areas of your body. During the scan you will be asked to lie very still on a couch inside a long tube for about 30 minutes. It is painless but can be uncomfortable, and some people feel a bit claustrophobic during the scan. It is also noisy, but you will be given earplugs or headphones.

Some people are given an injection of dye into a vein in the arm, but this usually does not cause any discomfort.

Angiogram This test involves an injection of a dye to show up the blood vessels in the brain. The dye is injected through a thin, flexible tube (catheter) that is inserted into an artery in the groin, arm or neck. A series of x-rays are taken. Your nurse or doctor will explain this test in more detail.

Biopsy To give an exact diagnosis, a sample of cells from the tumour is taken, then looked at under a microscope. The biopsy involves an operation. Your doctor will discuss with you whether this is necessary in your case, and exactly what the operation involves.

The treatment for haemangioblastoma depends on a number of factors, including your general health and the size and position of the tumour. The results of your tests will enable your doctor to decide on the best type of treatment for you.

There are some risks associated with treatment to the brain and your doctor will discuss these with you.

Your treatment will usually be planned by a team of specialists known as a multidisciplinary team (MDT). The team will usually include a doctor who operates on the brain (neurosurgeon), a doctor who specialises in treating illnesses of the brain (neurologist), a doctor who specialises in treating cancer (an oncologist), a specialist nurse and possibly other health professionals, such as a physiotherapist or a dietitian.

If the pressure in the skull is raised, it is important to reduce it before any treatment is given for brain tumours. Steroid drugs may be used to reduce swelling around the tumour. If raised intracranial pressure is due to a build-up of cerebral spinal fluid (CSF), a tube (shunt) may have to be inserted to drain off the excess fluid.

Consent

Before you have any treatment, your doctor will give you full information about what it involves and will explain its aims. They will usually ask you to sign a form saying that you give your permission (consent) for the hospital staff to give you the treatment. No medical treatment can be given without your consent.

Benefits and disadvantages of treatment

Treatment can be given for different reasons and the potential benefits will vary depending upon the individual situation. If you have been offered treatment that aims to cure your cancer, deciding whether to have the treatment may not be difficult. However, if a cure is not possible and the treatment is to control the cancer for a period of time, it may be more difficult to decide whether to go ahead.

If you feel that you can’t make a decision about the treatment when it is first explained to you, you can always ask for more time to decide.

You are free to choose not to have the treatment and the staff can explain what may happen if you do not have it. You don’t have to give a reason for not wanting to have treatment, but it can be helpful to let the staff know your concerns so that they can give you the best advice.

Surgery

Surgery is almost always used to treat haemangioblastoma. As the tumour does not spread to other parts of the brain, in most cases it is possible to remove it completely during an operation. However, some haemangioblastomas are difficult to reach with surgery – for example, in the lower part of the brain (brainstem) – and surgery may not then be possible. If this is the case your doctor will discuss other types of treatment with you.

Radiotherapy

Radiotherapy treatment uses high-energy rays to destroy the cancer cells, may be used after surgery if the tumour has not been removed completely. It may be used to treat haemangioblastoma if surgery is not possible.

Chemotherapy

This is the use of anti-cancer (cytotoxic) drugs which destroy cancer cells. Chemotherapy is rarely used for treating haemangioblastoma.

You may find the idea of a tumour affecting your brain extremely frightening. The brain controls the body, and not being in control is something that can be very worrying. You may experience many different emotions, including anxiety and fear. These are all normal reactions, and are part of the process that many people go through in trying to come to terms with their condition.

Many people find it helpful to talk things over with their doctor or nurse, or with one of our support service nurses. Close friends and family members can also offer support.

Driving

In some circumstances you may not be allowed to drive for a period of time. If you have had an epileptic fit, the Drivers and Vehicle Licensing Association (DVLA) will not allow you to drive for a year after your last fit. You can then drive again provided you remain well.

After surgery to the main part of the brain, the cerebrum, there is a small risk of epileptic fits and the DVLA requires that you do not drive for at least a year after this type of surgery.

You may not be allowed to drive some vehicles, such as a LGV (large goods vehicle) or a PCV (passenger carrying vehicle).

The hospital will not contact the DVLA, but it is your responsibility to do so and your doctor will advise you how to do this.

This section has been compiled using information from a number of reliable sources, including:

• Oxford Textbook of Oncology (2nd edition). Souhami et al. Oxford University Press, 2002.
• The Textbook of Uncommon Cancers (3rd edition). Raghavan et al. Wiley, 2006.
• Cancer in the Nervous System (2nd edition). Levin. Oxford University Press, 2002.

For further references, please see the general bibliography.