Craniopharyngiomas originate from epithelial cells of the craniopharyngeal canal, remnants of Rathke’s pouch, or through squamous metaplasia of residual epithelial cells from the primitive buccal cavity. Most tumors are located above the sella turcica, although a small number may be found within the sella itself. The condition typically presents in children and adolescents, although cases in adults are also observed. The onset is generally slow. Craniopharyngiomas can arise from any point within the pituitary-hypothalamic axis and develop along this pathway. The tumors are most commonly situated in the region between the sella turcica and the third ventricle. Approximately 50% of these tumors originate from the infundibulum and/or tuber cinereum at the base of the third ventricle and primarily expand towards the third ventricle. Obstruction of the foramina of Monro in the third ventricle may result in symptoms of increased intracranial pressure. Additionally, compression of the optic chiasm can lead to visual impairment and visual field defects.
If the tumor grows into the sella, compression of the pituitary gland may cause delayed growth, underdeveloped sexual organs, obesity, and dwarfism in children. In adults, symptoms may include sexual dysfunction and hypothalamic dysfunction, resulting in disturbances of temperature regulation and imbalances in water and electrolytes.
Craniopharyngiomas are histologically low-grade tumors (WHO Grade I) and are divided into two subtypes: adamantinomatous and squamous-papillary. The adamantinomatous subtype is more commonly observed in children, while the squamous-papillary subtype is more frequent in adults. Imaging often shows cystic degeneration and calcification within the tumor, with the normal pituitary tissue located at the bottom of the sella. Treatment primarily involves transnasal endoscopic removal of sellar and suprasellar tumors.