What is a Craniopharyngioma?
Craniopharyngiomas are rare, slow-growing tumours that develop near the pituitary gland at the base of the brain. The World Health Organization (WHO) classifies them as Grade I tumours, meaning they are not considered cancerous. However, they can still cause serious health problems because of their location near important brain structures.
Although benign, craniopharyngiomas can press on or closely involve nearby critical structures such as the pituitary gland, hypothalamus, optic nerves, blood vessels and the third ventricle. Because of this close proximity to important brain regions, the tumour’s location strongly influences the symptoms a person may experience, both at diagnosis and later in life.
Craniopharyngiomas can affect both children and adults. The two main subtypes, adamantinomatous and papillary craniopharyngioma, are more common in different age groups and have distinct biological features.
Types of Craniopharyngioma
Craniopharyngiomas are divided into two main subtypes based on their histology and molecular features: adamantinomatous craniopharyngioma (ACP), which occurs most commonly in children and papillary craniopharyngioma (PCP), which occurs almost exclusively in adults.
ACP is associated with a mutation in the CTNNB1 gene. This mutation causes abnormal accumulation of a protein called β-catenin inside tumour cells. This abnormal buildup activates signalling pathways involved in tumour growth and development.
PCP is strongly associated with the BRAF V600E mutation. This mutation activates signalling pathways that promote tumour development.
Craniopharyngioma Symptoms
Symptoms of craniopharyngioma depend largely on where the tumour develops in the brain. When it grows near the pituitary or hypothalamus, it can disrupt hormone regulation and affect growth, appetite, energy levels, and fluid balance. When it affects the optic chiasm (where the optic nerves cross near the base of the brain) it can cause visual problems.
Some of the early symptoms include headaches, nausea and vomiting, which may occur when the tumour blocks normal fluid flow and increases pressure inside the skull.
Other symptoms may include:
- Hormonal changes in children, such as slowed growth, changes in weight , and delayed puberty
- Endocrine-related symptoms such as fatigue, changes in appetite , excessive thirst, and increased urination
- Vision problems, including blurred vision, reduced peripheral vision or partial or complete vision loss
- Neurological and cognitive symptoms such as balance difficulties, memory or concentration problems, daytime sleepiness, or weakness
How Is Craniopharyngioma Diagnosed?
Craniopharyngiomas can be diagnosed after symptoms (usually hormone deficiencies or vision problems) have been present for some time, particularly when these early symptoms develop gradually.
Doctors usually investigate suspected craniopharyngiomas using imaging tests such as computed tomography (CT) and magnetic resonance imaging (MRI). Both scans are often used together because each provides different information. The two subtypes of craniopharyngioma can also appear different on imaging: adamantinomatous craniopharyngiomas (ACP) are often cystic and commonly contain calcifications, while papillary craniopharyngiomas (PCP) are usually more solid and rarely calcified.
CT scans are particularly useful for detecting calcifications, which helps in distinguishing ACP from PCP. MRI provides detailed information about the tumour’s size, relationship to nearby structures, and involvement of surrounding tissue. MRI’s are often performed using a contrast agent such as gadolinium to better define the tumour and its relationship to nearby structures.
Doctors may also perform hormone testing and detailed eye examinations to assess how the tumour is affecting pituitary function and vision.
Living With Craniopharyngioma
Although long-term survival rates for craniopharyngioma are generally high, many people continue to experience long-term effects that can significantly impact quality of life. This is largely because the tumour grows near critical brain structures such as the hypothalamus, pituitary gland, and optic chiasm. Both the tumour itself and the treatments used to control it can damage these areas.
Damage to the hypothalamus and pituitary gland can lead to long-term hormone deficiencies and problems regulating appetite, sleep, energy levels, and body weight. Many patients require lifelong hormone replacement therapy and ongoing medical care. These changes can also affect emotional well-being, behaviour, memory, and psychological health.
Supportive care may include hormone replacement therapy, psychological support, rehabilitation services, nutritional guidance, and physical activity programs tailored to the individual’s needs.
Craniopharyngioma Treatment and Management Options
Treatment for craniopharyngioma is tailored to each patient and may include surgery, radiotherapy, or a combination of both. When the tumour is located in an area that can be safely accessed, surgery is often the first treatment option.
Surgery for Craniopharyngioma
When safely possible, surgeons aim to remove as much of the tumour as they can while preserving nearby brain structures. Surgery may involve an endoscopic transnasal approach through the nose, which can provide a clearer and less invasive pathway to the tumour. In some cases, partial tumour removal followed by radiotherapy may reduce the risk of long-term complications, particularly when the tumour involves the hypothalamus. However, surgeons must take great care to avoid damaging nearby structures, particularly the hypothalamus. Injury to the hypothalamus during surgery can lead to complications such as significant weight gains, sleep disturbances, hormonal problems, fatigue, and difficulties with appetite regulation.
If complete removal is not possible, either because the tumour extends into nearby areas or is closely connected to critical brain structures, radiotherapy is often used after surgery to help control tumour growth. In some cases, surgery is also performed to reduce tumour size before radiotherapy, relieve symptoms, or restore normal flow of cerebrospinal fluid if it has been blocked by the tumour.
Radiotherapy and targeted treatments for craniopharyngioma
Radiotherapy is usually delivered using external beam techniques, including photon or proton therapy. In addition, doctors are increasingly exploring targeted therapies based on the genetic mutations found in craniopharyngioma, which may offer more precise treatment options with fewer side effects in the future.
For example, BRAF-targeted therapies have shown promising results in papillary craniopharyngioma. These distinct genetic changes are important because they open the door to more targeted treatments.
Clinical trials are now exploring therapies that specifically target these mutations, with the aim of making treatment more personalised and reducing treatment-related side effects.
Although craniopharyngioma is typically benign, its location near the pituitary gland means it can have lifelong effects on hormones, vision, and daily functioning.
For carers and families, support often involves long-term management. This could include coordinating medical care, managing behavioural or cognitive changes, and supporting ongoing health needs.
You don’t have to navigate this alone. Our Support Hub provides practical guidance, and our National Advocacy Service can help you access the support, services, and care your family needs over the long term.
References
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