Pineal region tumours

Pineal region tumours affect the pineal gland area of the brain. This gland produces a hormone called melatonin that helps control sleeping. They are divided into different tumour types depending on the kind of cells they develop from. The most common type is a germ cell tumour

Different tumours cause different symptoms. They may be due to a build-up of fluid in the brain, which leads to increased pressure, or caused by the tumour itself. They may include:

  • headaches
  • vomiting
  • sight problems
  • balance and coordination problems
  • hormonal imbalance

You will need to have some tests, which may include CT or MRI scan, biopsy, and lumbar puncture.

Your specialist will talk to you about the best treatment for you and explain the benefits and disadvantages. Because of their position, these tumours can be difficult to remove with surgery. You can have radiotherapy alone, after surgery or with chemotherapy. You usually have steroids to control your symptoms

Your doctors will explain what treatment side effects to expect and how they can be managed.

The pineal region

Pineal-region tumours are brain tumours that occur in the pineal region of the brain. You may also find our general information about brain tumours useful.

The pineal gland is found at the back of the third ventricle of the brain (see diagram below). 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', which controls when we sleep and when we wake. 

MACD031-Brain-cross-section-detail-labelled
MACD031-Brain-cross-section-detail-labelled

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Tumours of the pineal region

A tumour may be either benign (non-cancerous) or malignant (cancerous). 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 or spine. 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. About 4,700 people are diagnosed with brain tumours each year in the UK. About 1 in every 100 (1%) of these tumours are in the pineal region.

Brain cross-section
Brain cross-section

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Types of pineal-region tumours

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 germ cell tumour. This develops from germ cells which are cells in a very early stage of development. Germ cell tumours are divided into three main groups:

  • germinomas
  • nongermanomatous germ cell tumours
  • teratomas.

They are fast-growing and may often spread to other parts of the brain and spine. Other types of pineal tumour include:

  • astrocytomas
  • teratomas
  • meningiomaspineocytomaspineoblastomas.


Causes of pineal-region tumours

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


Signs and symptoms

Symptoms of tumours in the pineal region are usually due to increased pressure within the skull (raised intracranial pressure). This may be because of a blockage in the ventricles (fluid-filled spaces) 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 focusing 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.

Germ cell tumours in the pineal region may also involve the pituitary gland and cause delayed puberty in teenagers, or other hormonal difficulties.


Tests and investigations

For your doctors to 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 scan

A CT (computerised tomography) scan uses x-rays to build a three-dimensional picture of the inside of the body. You may be given either a drink or injection of dye. This is to make certain areas of the body show up more clearly. This scan takes around 30 minutes and is painless. We have more detailed information about having a CT scan.


MRI scan

This scan uses magnetism to build up a detailed picture of areas of your body. You may be given an injection of dye, into a vein, to improve the images from the scan. This test is painless and will take around 30 minutes. We have more detailed information about having an MRI scan.


Biopsy

To give an exact diagnosis, a sample of cells from the tumour (biopsy) is sometimes taken, and 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 whether a biopsy is necessary in your situation, and what the operation involves.


Blood tests

Blood tests for hormone levels will probably be taken, particularly if there are signs that the pituitary 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 CSF is then examined, as some nongermanomatous germ cell tumours produce distinctive chemicals that can be detected in it.

A lumbar puncture cannot be done if there is raised intracranial pressure, so it will probably be done later on in your treatment.


Treatment

The treatment for tumours in the pineal region depends on a number of things, including your general health and the size, type and position of the tumour. The results of your tests will enable you and your doctor to discuss the best treatment options.

Your treatment will usually involve a team of specialists known as a multidisciplinary team (MDT). The team will usually include:

  • a doctor who operates on the brain and spine (neurosurgeon)
  • a doctor who specialises in treating illnesses of the brain (neurologist)
  • a doctor who specialises in treating brain tumours (an oncologist)
  • a specialist nurse and possibly other healthcare professionals, such as a physiotherapist or dietitian.

If you have raised intracranial pressure it's important to reduce it before treatment begins. Steroid drugs may be given to reduce swelling around the tumour. If you have raised intracranial pressure because of 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.

Consent

Before you have any treatment, your doctor will give you full information about its aims and what it involves. They will 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 for each person. If you have been offered treatment that aims to cure your tumour, deciding whether to have the treatment may not be difficult. However, if a cure is not possible and the treatment is to control the tumour 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. Although you don’t have to give a reason for not wanting to have treatment, it can be helpful to let the staff know your concerns so that they can give you the best advice.


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 preferred form of treatment for some types of pineal tumours. The aim of surgery is to remove as much of the tumour as possible without damaging the surrounding brain tissue.

When surgery is not the most suitable treatment, radiotherapy may be given alone, or with chemotherapy, to treat pineal tumours.


Radiotherapy

Radiotherapy uses high-energy x-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.


Chemotherapy

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


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 types of vehicle, such as an LGV (large goods vehicle) or a PCV (passenger carrying vehicle).

The hospital will not contact the DVLA. It is your responsibility to do so and your doctor will advise you how to go about this.

You can contact the DVLA by phone on 0300 790 6806 or at dvla.gov.uk.


Back to Types of brain tumour

Acoustic neuromas

An acoustic neuroma is a of brain tumour of the hearing nerve. It is sometimes called a vestibular schwannoma.

Astrocytomas

Astrocytomas, also known as astrocytic tumours, are a type of brain tumour.

CNS lymphoma

Most lymphomas start in lymphatic organs. But CNS lymphoma starts in the central nervous system.

Ependymoma

An ependymoma is a rare type of brain tumour.

Haemangioblastoma

A haemangioblastoma is a rare brain tumour that starts in the cells that line blood vessels in the brain.

Medulloblastoma

A medulloblastoma is a rare form of brain tumour.

Meningioma

A meningioma is a brain tumour that starts in tissues called the meninges that protect the brain. Most are benign.

Mixed gliomas

Mixed gliomas are a type of brain tumour that contains different types of glial cells.

Oligodendroglioma

An oligodendroglioma is a type of brain tumour that starts in the cells that cover nerve cells.

Pituitary tumours

Pituitary tumours start in the pituitary gland, which is at the base of the brain. They are usually benign.