|
Incidence: 9% approx of all Childhood Intracranial Tumours Craniopharyngiomas are benign tumours accounting for between 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 symptomsCraniopharyngiomas 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 (more common 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 occasional precocious puberty.
- Thirst and passing large volumes of dilute urine (Diabetes Insipidus).
- Tired, difficult 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 14). InvestigationsAn 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 hypothalmic-pituitary axis), psychological and visual function. Treatment possibilitiesBecause of the position of the tumour, transcranial, subfrontal surgery is almost always required. However some small craniopharygiomas 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 teh ventricular system) is present. Patient managementAlmost 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 6), and it is relatively common for diabetes insipidus (factsheet 8) to develop. Children with panhypopituitarism may need long-term GH (factsheet 11), 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. WatchpointsUrgent - refer to hospital- Deterioration in vision and/or recurrent headache. These may indicate regrowth and/or cyst reoccurence.
- Intercurrent illness, in particular vomiting and diarrhoea in patients on cortiisol 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 9).
Non-urgent (but still very important)- Depression, psychological difficulties, problems in school are relatively common in chldren treated for craniopharyngioma.
Questions patients may askHow 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 post-operative 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 organisation 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 patientsPlease go to the Resource Library of this website to access resources for patients. Our library includes literuature, newsletters and articles available from The Pituitary Foundation as well as the contact information for other organisations who provide support and information. All of our patient leaflets are available to read as well as downloadable in PDF format from this website. Resources for GPsEndotext.org ‘Your Endocrine Source’
www.endotext.org (www.endotext.org/neuroendo/index.htm) Oxford Handbook of Endocrinology OUP (2002) JAH Wass & H Turner (Eds). More Specialist ResourcesManagement 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 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 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
|
Medical Information