ABSTRACT

Craniopharyngiomas are slow-growing, benign epithelial neoplasms of the sellar region that arise from embryonic squamous cells of the hypophysiopharyngeal duct (Rathke’s pouch). Although Erdheim originally described this lesion in 1904, in 1932, Cushing introduced the term ‘‘craniopharyngioma’’ to denote its origin from the embryological remnant. Histologically benign, these tumors nonetheless display insidious growth to involve important neurovascular structures. Endocrine, visual, and mental disturbances are frequently seen at presentation and are secondary to involvement of the hypothalamus-pituitary axis, optic pathways, thalamus, and frontal lobes. Early attempts at surgical resection were associated with a high risk of damage to the local neuroanatomy. Although advances in microsurgical and skull base techniques, radiation therapy, chemotherapy, and hormonal replacement have provided better long-term survival and longer recurrence-free intervals, controversies remain as to the optimal treatment of this tumor.