Clinical meaning
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).
Diagnosis & workup
Diagnostics & workup: - Brain MRI with contrast: mixed solid/cystic mass in sellar/suprasellar region; calcifications best seen on CT (90% of adamantinomatous type calcify) - Visual field testing: bitemporal hemianopia from optic chiasm compression (most common visual field defect) - Complete pituitary hormone panel: GH (growth failure in children), TSH/free T4, ACTH/cortisol (morning cortisol <3 = adrenal insufficiency), LH/FSH, prolactin, ADH (diabetes insipidus) - Water deprivation test: diagnose diabetes insipidus (failure to concentrate urine despite dehydration) - CT head: calcifications in sellar/suprasellar region (highly suggestive of adamantinomatous craniopharyngioma in children) - Formal ophthalmologic examination: visual acuity, visual fields, fundoscopy