This information is about brain tumours that occur in the pineal region of the brain. It should ideally be read with our general information about brain tumours.

The pineal gland is found at the back of the third ventricle of the brain (see diagram). Ventricles are fluid-filled spaces within the brain. The functions of the pineal gland are not fully understood, but one function is to produce the hormone melatonin. Melatonin is involved in regulating the body’s 'internal clock', controlling when we sleep and when we wake.

Cells within the brain normally grow in an orderly and controlled way, but if for some reason this order is disrupted, 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.

Pineal-region tumours can be made up of different types of cells.

Although this type of tumour is more common in adults, it can occur in children. For unknown reasons, it is more common in men than in women.

Tumours affecting the whole of the central nervous system (CNS), which is made up of the brain and spinal cord, are fairly rare. About 4500 new tumours are diagnosed each year in the UK. About one in every 100 (1%) of these tumours are in the pineal region.

Pineal-region tumours can be made up of different types of cells. The most common type of tumour in the pineal region is known as a germinoma. Germinomas develop from germ cells (cells in a very early stage of development). They are fast-growing and may often spread to other parts of the brain.

Other types of pineal tumour include:

• astrocytomas
• teratomas
• meningiomas
• pineocytomas
• pineoblastomas.

As with most brain tumours, the cause of these tumours is unknown. Research is being carried out into possible causes.

Symptoms of tumours in the pineal region are usually due to increased pressure within the skull (raised intracranial pressure). This may be due to a blockage in the ventricles of the brain, which leads to a build-up of cerebrospinal fluid (CSF). CSF is the fluid that surrounds and protects the brain and spinal cord. This increased pressure may also be caused by swelling from the tumour itself.

The first sign of this type of tumour in children is often hydrocephalus (water on the brain), which can cause enlargement of the skull.

Other symptoms are likely to be headaches, vomiting (sickness) and sight problems. Children are often tired and irritable. The tumour may cause difficulty in looking upwards, or focussing on close objects.

Symptoms may be caused by the tumour pressing on surrounding areas of the brain, such as the cerebellum. This will lead to problems with coordination and balance. People often have difficulty walking and may appear to stumble or walk awkwardly.

Tumours in the pineal region may extend to the pituitary gland and cause delayed puberty in teenagers, or other hormonal difficulties.

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 examination (nerve tests) You will usually have a neurological examination to assess any effect the tumour has on your nervous system.

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 but 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. A CT scan may be used to identify exactly where the tumour is or to check for any spread of the cancer.

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 the 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.

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

Blood tests Blood tests for hormone levels will probably be taken, particularly if there are signs that the pineal gland is affected.

Lumbar puncture A test called a lumbar puncture may be done. The skin on your back is numbed with local anaesthetic, and a needle is passed through the skin so that a small amount of CSF can be withdrawn for tests. The spinal fluid (CSF) is then examined, as some germinomas produce distinctive chemicals that can be detected in the CSF. This last test cannot be done if there is raised intracranial pressure so it will probably be done later on in your treatment.

The treatment for tumours in the pineal region depends on a number of things, 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 treatment for 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.

Before any treatment can be given for this type of brain tumour, it is important to reduce the pressure in the skull if it is raised. Steroid drugs may be given to reduce swelling around the tumour. If you have raised intracranial pressure due to a build-up of CSF, a tube (shunt) may have to be inserted into the brain to drain off the excess fluid. Alternatively, a minor operation called a third ventriculostomy can create a new drainage channel.

Surgery

The position of the pineal region, at the centre of the brain, often makes surgical removal of these tumours very difficult.

However, where possible, surgery is the first form of treatment for pineal tumours. The aim of surgery is to remove as much of the tumour as possible without damaging the surrounding brain tissue.

If surgery is not possible, the tumour may be treated with either radiotherapy or chemotherapy.

Radiotherapy

Radiotherapy treatment is the use of high-energy rays to destroy the cancer cells. It is often used to treat tumours in the pineal region, especially germinomas as they respond very well to radiotherapy.

Radiotherapy is often given after surgery to destroy any remaining cancer cells. If there are signs that the cancer has spread to the spine, radiotherapy will be given to the spinal cord as well as the brain. When surgery is not possible, radiotherapy may be given alone, or with chemotherapy, to treat pineal tumours.

Chemotherapy

Chemotherapy is the use of anti-cancer (cytotoxic) drugs that destroy cancer cells. Chemotherapy is often used, with radiotherapy, to treat germinomas. It is rarely used for other pineal tumours although research is looking at ways of developing this type of treatment.

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 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.

If you have 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 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 (2nd edition). Raghavan et al. Wiley, 1999.
• Cancer in the Nervous System (2nd edition). Levin, Oxford University Press, 2002.

For further references, please see the general bibliography.