Ependymoma

An ependymoma is a type of tumour that arises from the cells that line the fluid filled spaces of the brain and spine. 

An ependymoma can be classified as either malignant (cancerous) or benign, depending on how aggressive the tumour is. They can occur anywhere in the brain or spine, and have the potential to spread (disseminate or metastasise) to the spinal cord via the cerebrospinal fluid.

Less than 50 children a year develop ependymoma in the UK, representing around 10% of all childhood brain and spinal cord tumours.

Approximately 60% of all children diagnosed with this tumour type are less than five years old. Ependymomas can occur at any age but are rare among adults. Ependymomas are twice as likely to occur in the posterior fossa region of the brain which is found at the lower back of the head. Like most brain tumours the cause of ependymoma is unknown.

Signs and symptoms

The symptoms of ependymoma depend on where the tumour is located and whether there is increased pressure in the head. This is called raised intracranial pressure. Common symptoms may include:

  • nausea and vomiting
  • lethargy and irritability
  • headaches
  • clumsiness
  • difficulty with tasks like handwriting
  • gradual decline in school performance
  • changes in personality and behaviour
  • altered way of walking.

If the tumour spreads or is located in the spinal cord, the signs and symptoms may include:

  • back pain
  • difficulty walking
  • problems with bowel and bladder control.

Tests and investigations

Doctors will need to carry out some tests to find out as much as possible about the type, position and size of the tumour. This will help them decide on the best treatment. These tests include:

  • CT scan
  • MRI scan
  • lumbar puncture.

Staging

Staging is a measure of how far the tumour has spread beyond its original site. The most important features in ependymoma are whether the tumour has spread to the spinal canal and whether the neurosurgeon has removed the entire tumour.

Ependymoma prognosis

The prognosis for ependymoma is very closely related to whether the neurosurgeon can remove all of the tumour, and the spread and aggressiveness of the tumour.

Children who have had an incomplete removal of tumour at initial surgery, or evidence of tumour spread, have a reduced survival rate. The treatment of infants is difficult but results with intensive chemotherapy have been very encouraging.

Long-term effects

As ependymoma can occur in young children and the treatment is given at an important time of the child's development there may be some long-term effects of treatment. These could include:

  • growth and hormonal changes
  • reduced fertility
  • behavioural changes
  • possible learning problems
  • difficulties with coordination.
  • hearing and visual disturbances.

There is a small risk of developing a second cancer later in life.

Follow-up care

All children treated for ependymoma are followed up after treatment is finished in the long-term brain tumour follow-up clinic. Your child will be seen at regular intervals in this clinic, indefinitely, so that we can help with any long-term effects of the treatment.