Overview
Clinical Meaning
Craniopharyngioma is a benign but locally aggressive epithelial tumor arising from Rathke pouch remnants along the craniopharyngeal duct.
AGPCNP
AGPCNP
Craniopharyngioma is a benign but locally aggressive epithelial tumor arising from Rathke pouch remnants along the craniopharyngeal duct. Two histologic subtypes exist: adamantinomatous (children/adolescents, calcifications, cystic with machinery oil-like fluid, CTNNB1 beta-catenin mutations) and papillary (adults, solid, rarely calcified, BRAF V600E mutations). These tumors grow in the sellar/suprasellar region, compressing the optic chiasm (bitemporal hemianopia), pituitary gland (hypopituitarism), hypothalamus (obesity, temperature dysregulation, behavioral changes), and third ventricle (obstructive hydrocephalus). Despite being histologically benign (WHO Grade I), they cause significant morbidity due to their critical location and high recurrence rate (20-50% after surgery).
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