Craniopharyngioma

Craniopharyngiomas are WHO grade 1 epithelial tumors of the sellar and suprasellar region, thought to arise from remnants of Rathke's pouch. There are two main types: adamantinomatous, seen in children and adults and associated with CTNNB1 mutations, and papillary, seen mostly in adults and associated with BRAF V600E mutations.

Although histologically benign, they sit against the pituitary stalk, hypothalamus, optic chiasm and the third ventricle, so presenting symptoms include visual loss, hormonal deficiency, headache and hydrocephalus.

Treatment balances tumor control against injury to these structures. Gross-total resection lowers recurrence but increases the risk of permanent diabetes insipidus and other endocrine deficits; subtotal resection followed by radiation is a common alternative. BRAF-mutant papillary tumors may respond to targeted therapy.