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Factsheet 8 Craniopharyngiomas are benign tumours accounting for approximately 9% of all intracranial tumours in childhood, and are the most common peripituitary tumour in this age group. In adults, craniopharyngiomas account for 1% of brain tumours. Their behaviour is unpredictable and their origin is not fully understood. They are usually situated close to the Pituitary gland and Hypothalamus. The tumour consists of solid parts, sometimes calcified, and cysts, which may be filled with a dense oily fluid. PRESENTING SYMPTOMS
Craniopharyngiomas can cause symptoms at any age, but those presenting in childhood are relatively fast growing, have a more aggressive course and can be more difficult to treat. Symptoms have usually been present for some time before diagnosis. They are usually associated with raised intracranial pressure and possibly anterior and posterior pituitary failure. Symptoms and signs at presentation may include: - Headaches (typically in the morning), nausea and vomiting (commoner in children).
- Visual field defect (typically bilateral temporal hemianopia - poor vision to both sides - but complete loss of vision in one or both eyes can occur).
- Slow growth during childhood.
- Delayed or arrested puberty, and very occasionally precocious puberty.
- Thirst and passing large volumes of dilute urine (Diabetes Insipidus).
- Tired, difficulty recovering from minor illness (ACTH, Cortisol deficiency).
- Tired, cold intolerance, constipation, slow pulse, dry skin (TSH/T4 deficiency).
- Changes in behaviour.
Please see ‘Who and When to Refer’ (factsheet 15). INVESTIGATIONS
An MRI or CT scan determines the precise size and position. Additional tests that form part of the evaluation include assessments of biochemical, endocrine (especially an assessment of hypothalamic-pituitary axis), psychological and visual function. TREATMENT POSSIBILITIES
Because of the position of the tumour, transcranial, subfrontal surgery is almost always required. However some small craniopharyngiomas may be approached by the transsphenoidal route. Complete excision is rare. In most patients, partial removal is followed by Radiotherapy. Prior to the operation, or if the tumour re-grows, insertion of a CSF shunt may be required if hydrocephalus (excessive accumulation of CSF in the ventricular system) is present. PATIENT MANAGEMENT
All patients require long-term follow up, to allow early detection of any tumour re-growth and to treat the effects of the lesion on hypothalamic and pituitary function. Recurrence after surgery alone is common, but is less likely when radiotherapy is also given. Following surgery, at approximately 2-3 months, a further assessment of hypothalamic-pituitary axis is required. Many patients will be panhypopituitary (factsheet 7), and it is relatively common for diabetes insipidus (factsheet 9) to develop. Children with panhypopituitarism may need long-term GH (factsheet 12), cortisol, thyroid and sex hormone replacement. Annual MRI or CT scans to check for re-growth are especially important in the first 3 years after treatment. Other common problems post-op are increased or decreased appetite (resulting in weight problems) and reduced powers of concentration and short-term memory loss. WATCHPOINTS
Urgent - refer to hospital
- Deterioration in vision and/or recurrent headache. These may indicate regrowth and/or cyst recurrence.
- Intercurrent illness, in particular vomiting and diarrhoea in patients on cortisol replacement. Hydrocortisone replacement will need to be increased, and may need to be given by injection. Families should have hydrocortisone injections available for emergency use.
- CSF leak (very rare in children) Clear fluid dripping down the back of the throat or through the nose soon after surgery (factsheet 10)
Non-urgent (but still very important) - Depression, psychological difficulties and problems in school are relatively common in children treated for Craniopharyngioma.
QUESTIONS PATIENTS MAY ASK How will I know if the craniopharyngioma is re-growing?
There may be no symptoms. Regular MRI scans are essential. How likely is it that the craniopharyngioma will re-grow?
If this is going to occur then the majority of cases are within 3 years, but it may recur many years later. Partial removal results in a higher chance of the tumour increasing in size. This risk is considerably reduced by postoperative radiotherapy. Will my child be able to continue at school?
Most children remain in mainstream schooling without experiencing any difficulties. Difficulties may include sight problems, psychological and educational difficulties. Are my other children likely to develop this?
No, it is not hereditary or familial. Will it be possible for my child to have children?
With appropriate treatment at the time fertility is required, the young person should have a good chance of achieving fertility. Why is my child so sleepy?
As these tumours tend to occur above the pituitary, the part of the brain that deals with the organization of sleep and wakefulness is sometimes disturbed as the tumour grows, or by the surgical treatment. Damage to this part of the brain is often associated with increased somnolence. Why has my child’s appetite increased?
The part of the brain that deals with feeling full after eating is sometimes disturbed for the same reasons
RESOURCES FOR PATIENTS FOR GPs MORE SPECIALIST RESOURCES - Management of Pituitary Tumors: The Clinician’s Practical Guide (2003) (Second Edition), Editors and authors Michael P. Powell, Stafford L. Lightman and Edward R. Laws. Humana Press, Totowa, New Jersey
- Pituitary Tumours. Recommendations for service provision and guidelines for management of patients. Consensus statement of a working party (1997) RN Clayton & JAH Wass (Eds) London: Royal College of Physicians
- The Diagnosis and Treatment of Pituitary Insufficiency (1997) Lamberts SWJ (Ed) Bristol, UK: BioScientifica
- Endocrinology (1997) Levy A & Lightman SL New York: Oxford University Press
- The Epidemiology, Pathogenesis and Management of Pituitary Tumours (1998) Webb SM (Ed) Bristol, UK: BioScientifica
©2011 Version 3 (To be reviewed by 2013)
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