23rd May 2025

Craniopharyngioma

Craniopharyngiomas are benign non-glial epithelial tumours arising from remnants of Rathke’s pouch. These tumours often grow above the pituitary gland in the sellar/suprasellar space adjacent to the optic chiasm and often lead to a deficiency of anterior pituitary hormones. They account for 5-10% brain tumours in children and 1-3% of all adult intracranial tumours. The aetiology is unknown. 

Classification

Craniopharyngiomas are classified based on pathology:

• Adamantinous type (>80%): Adamantinous epithelium, keratin nodules, poorly demarcated from brain tissue, associated withs CTNNB1 gene mutation
• Squamous papillary type (<20% of tumours): Squamous epithelium with papillaries, well demarcated from brain tissue, rare in children, associate with BRAF oncogene mutation

Clinical Features

• Visual disturbances 
• Headache
• Intracranial hypertension: nausea, vomiting, double vision
• Endocrine: Symptoms of hypogonadotrophic hypogonadism (as listed above, galactorrhoea 
• Paediatric: macrocephaly and hydrocephalus (prior to fusion of intracranial sutures) and growth failure

Investigations

• Visual field testing
• MRI brain with contrast
• Serum: PRL, GH, IGF-1, LH, FSH, TSH, morning testosterone, morning ACTH and cortisol, U&Es
• Paired urine serum osmolality 
• Can consider tissue histology following surgical biopsy/resection

Image Source: Wikimedia Commons (2011). Craniopharyngioma, Licensed under a creative commons licence. Available for free at: https://commons.wikimedia.org/w/index.php?curid=15959020 (Accessed 13 May 2025).

Management

• The exact type of surgical management (biopsy only, cyst aspiration, CSF shunting, or tumour resection) depends on tumour size and location
• Post-operative ophthalmology review will be required
• Hormonal replacement therapy 
• Follow-up is done every 6-12 months and involves MRI brain scans as well as endocrinology review 

Complications

• Pituitary hormone deficiencies
• Diabetes insipidus 
• Visual impairment 
• Rarer complications include: seizures, hypothalamic injury, cognitive dysfunction