Craniopharyngioma: symptoms, diagnosis and treatment

A craniopharyngioma is a slow-growing tumour at the base of the brain, in the area just above the pituitary gland. The pituitary is a pea-sized gland that makes hormones controlling many body functions. Cancer Research UK explains that craniopharyngiomas are rare and can affect both children and adults.

The Pituitary Foundation describes the tumour as a rare cystic or solid mass that puts pressure on the hypothalamus, the part of the brain just above the pituitary that controls when pituitary hormones are released. The tumours can be solid, cystic (filled with fluid), calcified or full of debris.

Although the NHS groups non-cancerous tumours as "low grade" (grade 1 or 2) and notes that they grow slowly, a tumour in this position can still cause serious problems because it sits close to the optic nerves, pituitary and hypothalamus.

Cancer Research UK says symptoms can develop suddenly, so that you feel unwell quickly, or more slowly over time. The Pituitary Foundation adds that these are slow-growing tumours that can take 2 to 3 years or longer to show themselves before a diagnosis is made, and that you may not have all of the symptoms listed.

Symptoms come from three directions: pressure on nearby structures, pressure building up in the head, and loss of hormone production.

• Headaches, sometimes with nausea or vomiting.

• Changes to eyesight or other vision disturbance.

• Signs of raised pressure in and around the brain: double vision, feeling or being sick, sleepiness and confusion, and problems with balance and coordination.

• Increased thirst and passing a lot of urine, from a form of diabetes (AVP deficiency, previously called diabetes insipidus).

• Disturbed sleep, appetite and weight changes, and sensitivity to cold or heat.

• In children: slow growth and early or delayed puberty.

• In adults: irregular or absent periods, reduced fertility, lower sex drive, difficulty getting an erection, and tiredness with susceptibility to infection.

• Behavioural changes, including becoming more withdrawn or finding it hard to concentrate.

The Pituitary Foundation says about half of cases occur in children under 16 and that faster-growing tumours tend to affect children, while slower-growing ones affect adults. Cancer Research UK describes two types: adamantinomatous craniopharyngioma, mostly found in children and teenagers and in most adults, and papillary craniopharyngioma, which mainly occurs in adults.

In children the effects on growth and puberty are often an early sign, because the tumour interferes with the hormones that drive both. In adults, the first clues are more likely to be tiredness, changes to periods or sex drive, or vision change.

The Pituitary Foundation describes this type of tumour as congenital, meaning it is present from birth, even though it may not cause symptoms for years. None of the UK sources read for this page identify a lifestyle, infection or environmental cause, and nothing you did is known to bring one on.

The NHS notes more generally that the cause of most brain tumours is unknown. Because craniopharyngiomas are rare, you should not expect a cause to be found in an individual case.

The NHS says that if a GP cannot identify a more likely cause of symptoms such as persistent headaches or vision problems, they may refer you to a neurologist for assessment and tests such as a brain scan. Pituitary-related symptoms may instead lead to an endocrinologist.

Cancer Research UK lists the tests you might have: blood tests for hormone levels, an MRI scan or CT scan, eye examinations and a biopsy. The Pituitary Foundation gives a similar list of CT, MRI, an ophthalmic assessment and an endocrine assessment of blood hormone levels. The tests show the size, type and position of the tumour and how much it has affected hormones and vision, which helps the team plan treatment.

Cancer Research UK says most people have surgery. A neurosurgeon removes all of the tumour or just part of it, depending on where it is. The Pituitary Foundation explains that the surgeon will try to remove the tumour if possible, but surgery can damage nearby structures, so some of the tumour is often left behind on purpose to reduce that risk.

Cancer Research UK describes two ways of reaching the tumour: through the nose (transsphenoidal surgery), or through a small opening in the skull. Tumours close to the optic nerve and hypothalamus cannot usually be removed completely.

Radiotherapy may be given after surgery if the tumour was not completely removed, or if it comes back. For small tumours, stereotactic radiotherapy or radiosurgery targets high doses at the tumour. Cancer Research UK also notes that some people may have proton beam therapy.

Hormone replacement is often needed, either because of the tumour itself or as a side effect of treatment. The type depends on which hormones are low. After surgery, the Pituitary Foundation says hormone levels are rechecked and replacement given if they are depleted.

Cancer Research UK lists possible effects after the operation: changes in hormone levels, a type of diabetes that causes thirst and a lot of urine, changes in vision, weight gain and changes in behaviour. Because the tumour and its treatment sit so close to the pituitary and hypothalamus, long-term hormone replacement is common.

The Pituitary Foundation notes that appetite and weight variations, sleep disturbance and sensitivity to heat or cold can all be part of the picture. Specialist endocrine follow-up is how these are managed.

Cancer Research UK says you will have regular appointments after treatment. At each one the doctor examines you and asks about symptoms and side effects. Some visits include hormone tests, eye tests or MRI scans. Check-ups are generally more frequent at first and then depend on your situation.

The Pituitary Foundation says a follow-up MRI may be needed to make sure the tumour is stable, especially if it could not be removed completely. Contact your doctor or specialist nurse if you notice new symptoms; you do not need to wait for the next scheduled visit.

See a GP if you have headaches that feel different from your usual ones or are getting worse, or if you have vision or speech problems, seizures or persistent sickness. The NHS says these symptoms may not mean a brain tumour but should be checked.

The Pituitary Foundation describes adrenal crisis as a life-threatening drop in cortisol, with common triggers including sudden diarrhoea or vomiting, a temperature above 38 degrees C, significant injury or major psychological stress. If you take steroid replacement, carry the NHS steroid card and know your sick day rules. Call 999 if you or someone with pituitary hormone deficiency collapses or is seriously unwell.

Cancer Research UK says pressure in the head can cause double vision, feeling or being sick, sleepiness and confusion. Sudden severe headache, rapidly worsening vision, drowsiness or confusion need emergency assessment: call 999 or go to A&E.

In the NHS, a suspected craniopharyngioma is usually picked up through a GP, optician or hospital scan and then handled by a specialist team including a neurosurgeon, an endocrinologist, an eye specialist and an oncologist. Because the condition is rare, treatment is usually concentrated in a few specialist centres rather than every hospital.

Private care can offer quicker appointments or scans in some places, but the major treatment decisions are generally still made by the same type of specialist team. If you are considering private care, ask who will manage hormone replacement and follow-up over the following years, as this is where much of the long-term care lies.

Cancer Research UK points out that coping with a rare brain tumour can be difficult practically and emotionally, and that being well informed about the type you have and its treatment can make it easier. It signposts its information nurses on freephone 0808 800 4040, open 9am to 5pm Monday to Friday.

The Pituitary Foundation offers information on topics such as weight control, driving, prescription charges and benefits for people with pituitary conditions, alongside helplines and support groups.