Craniopharyngioma is a rare, benign brain tumor arising from embryonic pituitary rest cells near Rathke's pouch. Accounts for 2-5% of primary brain tumors (bimodal age 5-14 & 50-74).
Tumor Subtypes (ACP vs PCP)
1. Adamantinomatous Craniopharyngioma (ACP): Predominantly affects children; cystic with solid areas; calcifications present in ~90% of cases.
2. Papillary Craniopharyngioma (PCP): Found almost exclusively in adults; solid composition; rare calcification; almost all contain BRAF gene mutations.
3. Mixed / Transitional Subtype.
Location & Clinical Impact
Suprasellar Region (95% of cases): Compresses optic chiasm (bitemporal hemianopsia vision loss in 62-84%), hypothalamus, and pituitary stalk (causing polydipsia, polyuria, cortisol/thyroid/growth hormone deficits, and hydrocephalus ICP).
Diagnostics, Surgery & Hormone Therapy
Diagnostics: MRI (gold standard for tumor size & cystic component), CT scans (detects calcifications), Visual Field Testing (perimetry), and Endocrine blood panels.
Surgery: Endoscopic Endonasal Transsphenoidal (EET) or Transcranial approaches guided by Puget & Kassam classification systems.
Therapies: Stereotactic radiosurgery, Brachytherapy, Intracystic chemo, BRAF-targeted therapy for PCP, and lifelong post-op hormone replacement (hydrocortisone, levothyroxine).