Benign non-glial epithelial central nervous system tumour, constituting 1% to 3% of intracranial tumours in adults, most often located near the optic chiasm.
Bimodal age incidence with 50% in children aged between 5 and 14 years and a second peak in adults 50 to 70 years of age.
Common acute presentations are sudden visual loss and symptoms of intracranial hypertension.
Pituitary dysfunction is also common; children may present with growth failure and adults with diabetes insipidus and sexual dysfunction.
Primary treatment consists of surgery. Following surgery, radiotherapy may be required.
The majority of patients require adjunctive endocrine replacement therapy.
Craniopharyngiomas are benign, extra-axial, non-glial epithelial tumours of the central nervous system and are seen in both children and adults. They most commonly arise within the sellar/suprasellar space. Clinically, craniopharyngiomas cause mass effect symptoms, including visual loss or symptoms of intracranial hypertension. In addition, pituitary dysfunction is common and the majority of patients will have an associated endocrinopathy.
History and exam
Key diagnostic factors
- visual loss
- macrocephaly and hydrocephalus
- growth failure
Other diagnostic factors
- symptoms of hypogonadotrophic hypogonadism (amenorrhoea, erectile dysfunction)
- symptoms of intracranial hypertension (nausea, vomiting, decreased sensorium, diplopia)
- optic atrophy
- age 5 to 14 years
- age 50 to 70 years
1st investigations to order
- ophthalmological evaluation; computerised visual-field examination
- MRI brain (contrast-enhanced)
- CT brain (contrast-enhanced)
- serum prolactin
- serum growth hormone (GH)
- serum insulin-like growth factor 1 (IGF-1)
- serum insulin-like growth factor binding protein-3 (IGFBP-3)
- provocative growth hormone (GH) tests
- serum luteinising hormone
- serum follicle-stimulating hormone
- morning serum testosterone
- serum thyroid-stimulating hormone and T3/T4
- morning serum cortisol and adrenocorticotrophic hormone (ACTH)
- serum electrolytes
- urine and serum osmolality
- urine specific gravity
- plain x-rays for bone age
Investigations to consider
- tissue histology
at initial treatment
post initial treatment
- Pituitary adenoma
- Diaphragmatic meningioma
- Chiasmatic epidermoid
- Long term follow up of survivors of childhood cancer
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