A craniopharyngioma is a tumour which generally occurs above the pituitary gland. Located at the bottom of the brain, the gland is about the size of a pea and controls many vital functions.

Craniopharyngiomas result from the growth of cells that, early in foetal development, have failed to migrate to their usual area.

Craniopharyngiomas are benign tumours that do not spread; they can be solid, cystic (full of fluid), calcified or full of debris. They are slow-growing tumours that can take two to three years or longer to manifest themselves before a diagnosis is made. They can cause serious problems by interfering with important structures near them.

Craniopharyngiomas represent 5–10% of childhood brain tumours, with about 30 cases a year in children in the UK. They also occur in adults. There is no known cause.

Signs and symptoms

Symptoms result either from the tumour blocking the flow of fluid surrounding the brain (resulting in increased intracranial pressure) or from direct pressure and damage on the pituitary gland or optic nerves. Symptoms may include:

  • headaches and nausea or vomiting
  • hormone disturbances
  • disturbed sleep patterns
  • visual disturbance
  • behavioural changes
  • slow growth
  • increased sensitivity to cold or heat
  • early or delayed puberty
  • appetite and weight variations.

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 for your child. These tests include:

  • CT scan
  • MRI scan
  • ophthalmic assessment
  • endocrine assessment.

Treatment of craniopharyngioma

Surgery is the main treatment strategy for craniopharyngioma, and if possible the neurosurgeon will try to remove the tumour.

However, surgery can cause significant damage to the surrounding brain, and often the tumour will be left behind intentionally to avoid further damage to the child, and radiotherapy administered to stop the tumour from growing.

Craniopharyngioma prognosis

Most children with craniopharyngioma are cured by surgery and radiotherapy, but many suffer from serious long-term problems, such as hormone deficiencies, some of which can be life shortening.

What are the possible long-term effects?

Craniopharyngiomas, although benign, have many potential serious effects on a child’s health and development. These include growth, hormonal and behavioural changes, and learning difficulties. Visual disturbances can also result from both the tumour and its treatment.

A specialist hormone doctor (endocrinologist) will need to be involved in the long-term care of patients. They will replace hormones that are deficient, either as tablets or injections. The child may need urgent medical attention if they become unwell as they may not be able to control fluid balance and blood pressure normally.

Children can develop severe obesity problems due to hormone problems and damage to part of the brain that controls appetite.

Craniopharyngiomas need complex multidisciplinary management and can be very difficult to control. Behavioural problems and education difficulties can be severe and all children 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.