Craniopharyngioma

Abstract

Craniopharyngioma (CP) is a rare, benign, slow-growing, but clinically aggressive tumor located mainly in the sellar and suprasellar regions. While it occurs equally in children and adults, there are two peaks in the age distribution: first in 5–14 years of age and second in 45–74 years of age. The clinical presentation varies according to the age of patients, while the predominant symptoms are visual disturbances, headache, and endocrine dysfunctions. CPs are topographically classified in several subgroups based on the relationship of the tumor to the sella, diaphragma sellae, optic chiasm, stalk, and third ventricle; whereas the pathological classification includes two types: adamantinomatous (aCP) and papillary (pCP). Distinctive features of aCP are cysts with content of “motor-oil” fluid, calcification, wet keratin, peripheral palisading of basal cells, stellate reticulum, and mutations in CTNNB1/β-catenin gene; and those of Pcp are regular stratified squamous epithelium, devoid of cilia, papillary projections, no calcification, rare cyst with a clear fluid, and mutations in BRAF V600E. The surgical approaches include transcranial (subfrontal, pterional, transcallosal, and transcortical-transventricular) and transsfenoidal approaches, having different selection criteria, advantages, and disadvantages. Despite complete resection and radiotherapy, CPs are inclined to recur causing high morbidity and mortality.