Abstract
Craniopharyngioma is a benign epithelial neoplasm of the sellar region presumably derived from Rathke pouch epithelium.Two clinicopathological types are distinguished, the adamantinomatous and the (squamous) papillary type. The former shows odontogenic epithelial differentiation and is characterized by CTNNB1 mutations resulting in nuclear expression of beta-catenin.The latter occurs exclusively in adults and shows BRAF V600E mutation. Treatment of craniopharyngioma, a deep-seated benign tumor, has been one of the biggest challenges confronting neurosurgeons. Extent of surgical resection is the most critical prognostic factor for recurrence-free and overall survival. Recently, endoscopic endonasal surgery has become more broadly indicated because of its many advantages including direct and wide exposure of the tumor and surrounding vital structures, and avoidance of brain retraction. Despite significant improvements in overall survival, many “cured” patients, particularly pediatric patients, suffer from morbidities including endocrinopathy, visual impairment, hypothalamic dysfunction (obesity), neurocognitive dysfunction, et al. Multi-disciplinary and life-long management is essential.
