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Craniopharyngioma


Craniopharyngioma is a rare, usually benign (non-cancerous), slow-growing tumour that develops near the pituitary gland and hypothalamus. Although it does not usually spread to other parts of the body, its location means it can affect the pituitary gland, hypothalamus, optic nerves and other structures in the brain. This can result in problems with hormone production, vision, growth, appetite, thirst, sleep and other functions. (Orpha⁠)
 

How common is craniopharyngioma in Ireland?


Craniopharyngioma is very rare.


At present, there are no reliable national statistics available for the number of people living with craniopharyngioma in the Republic of Ireland. Ireland does not currently have a dedicated national patient registry for craniopharyngioma, meaning that the true number of people affected is not known.

International estimates indicate that craniopharyngioma is rare, with Orphanet reporting an estimated point prevalence of approximately 1 in 50,000 people internationally. This figure should not be interpreted as an estimate of the number of people living with craniopharyngioma in Ireland. (Orpha⁠)

Craniopharyngiomas can occur in both children and adults, with some types more commonly diagnosed during childhood and others more frequently seen in adults. (Orpha⁠)
 

What is craniopharyngioma?


A craniopharyngioma develops close to the pituitary gland and hypothalamus, areas of the brain that play an important role in controlling hormones and many essential body functions.

The tumour may be solid, cystic (fluid-filled), or contain both solid and cystic components. Some tumours may also contain calcification.

Because of its location, even a benign craniopharyngioma can place pressure on nearby structures. This can affect the pituitary gland and lead to deficiencies in one or more hormones. It can also affect vision and the hypothalamus, which is involved in regulating appetite, thirst, body temperature, sleep and other functions. (Cancer.gov⁠)

Craniopharyngiomas can develop in people of different ages. Symptoms may develop gradually and, in some cases, may be present for a considerable period before the condition is diagnosed. (Orpha⁠)

Symptoms

Symptoms can vary considerably depending on the size and location of the tumour and the structures affected.

You may experience some of the following symptoms, but not everyone will experience all of them:


  • Headaches, sometimes accompanied by nausea or vomiting
  • Changes or disturbances in vision
  • Hormone deficiencies caused by damage to the pituitary gland
  • Arginine Vasopressin Deficiency (AVP-D), formerly known as diabetes insipidus
  • Increased thirst and/or increased urination
  • Disturbed sleep patterns
  • Tiredness and reduced energy
  • Changes in appetite
  • Unexplained weight gain or weight changes
  • Problems regulating body temperature
  • Changes in behaviour, mood, concentration or memory
  • Slow growth or reduced growth in children
  • Delayed or early puberty in children
  • Reduced sex drive
  • Irregular or absent menstrual periods
  • Reduced fertility
  • Erectile difficulties in men
  • Growth hormone deficiency
  • Other pituitary hormone deficiencies


Children may also experience changes in growth, puberty, appetite and development. Adults may develop symptoms related to pituitary hormone deficiencies, vision changes or effects on the hypothalamus. (Orpha⁠)


Diagnosis


If craniopharyngioma is suspected, healthcare professionals may carry out a number of investigations to determine whether a tumour is present and to understand its size, location and effects on surrounding structures.


Investigations may include:


  • MRI scan – provides detailed images of the brain, pituitary region and surrounding structures.
  • CT scan – may be used to provide additional information about the tumour, including possible calcification.
  • Visual assessment – including formal visual-field testing where appropriate.
  • Endocrine assessment – blood tests are used to assess pituitary and other hormone levels.
  • Neurological assessment – to assess symptoms and the possible effects of the tumour.
  • Pathological examination – where tissue is obtained during surgery, examination can help confirm the type of craniopharyngioma.


MRI and CT imaging, together with endocrine and visual assessments, are important parts of the diagnostic process. (Cancer.gov⁠)


Treatment


Treatment for craniopharyngioma is individualised and depends on factors including the size and location of the tumour, the structures affected, the person's age and overall health, and whether the tumour can be safely removed.


Surgery


Surgery is an important treatment option for many people with craniopharyngioma.

Where it is considered safe, a neurosurgeon may attempt to remove as much of the tumour as possible. However, craniopharyngiomas can be closely associated with important structures including the pituitary gland, hypothalamus and optic nerves.


For this reason, completely removing the tumour may not always be possible or advisable. In some circumstances, leaving part of the tumour behind can reduce the risk of damage to surrounding structures. (Cancer.gov⁠)


Radiotherapy


Radiotherapy may be recommended when tumour tissue remains after surgery or if the tumour grows again. The type and timing of radiotherapy will depend on the individual circumstances.


Monitoring


In some situations, particularly where the tumour is small or stable and symptoms are limited, careful monitoring with regular MRI scans may be recommended.

Follow-up is important because craniopharyngiomas can recur or change over time. (Cancer.gov⁠)

Hormone replacement and long-term care

Because craniopharyngiomas develop close to the pituitary gland and hypothalamus, treatment or the tumour itself can affect hormone production.

Some people may develop one or more pituitary hormone deficiencies and require lifelong hormone replacement therapy.


This may include treatment for deficiencies involving:


  • Adrenal hormones
  • Thyroid hormones
  • Growth hormone
  • Sex hormones
  • Arginine Vasopressin (AVP)


AVP deficiency can affect the body's ability to regulate fluid balance and may cause excessive thirst and urination.

People who develop pituitary hormone deficiencies may require ongoing care from an endocrinology team, sometimes alongside other specialists such as neurosurgeons, ophthalmologists, neurologists and rehabilitation professionals.

Hormone deficiencies following craniopharyngioma and its treatment can be permanent and require careful long-term management. (Orpha⁠)


Living with craniopharyngioma


Living with craniopharyngioma can involve more than monitoring the tumour itself. Some people experience long-term effects on hormone production, vision, energy levels, weight, sleep, appetite, thirst, concentration, memory and emotional wellbeing.

The effects can vary significantly from one person to another.

Ongoing follow-up and access to appropriate specialist care can help manage the long-term effects of both the tumour and its treatment.

If you or someone you care for has been diagnosed with craniopharyngioma, it is important to discuss your individual diagnosis, treatment and follow-up plan with your healthcare team.


Craniopharyngioma and Pituitary Ireland


At Pituitary Ireland, we recognise that living with a rare pituitary or hypothalamic condition can be challenging for individuals and families.

Although there is currently no national registry providing reliable statistics on craniopharyngioma in the Republic of Ireland, we are committed to raising awareness of rare pituitary conditions and supporting people affected by them.

We aim to provide reliable, accessible information and connect people with appropriate sources of support.

Please remember: The information on this page is provided for general information and awareness purposes. It is not intended to replace medical advice, diagnosis or treatment from your healthcare professional.

Find out more

For additional information and support, you may wish to explore:

Craniopharyngioma is a rare condition, and no two people's experiences are exactly the same. If you have concerns about symptoms, treatment or long-term hormone replacement, please speak with your healthcare team.