Craniopharyngiomas: Natural History and Clinical Presentation

Abstract

Craniopharyngiomas are rare epithelial tumours arising along the path of the craniopharyngeal duct and account for 2–5 % of all the primary intracranial neoplasms and for up to 15 % of the intracranial tumours in children. The majority (94–95 %) has a suprasellar component. The potential proximity to and the subsequent pressure effects of craniopharyngiomas on vital structures of the brain (visual pathways, brain parenchyma, ventricular system, major blood vessels and hypothalamo-pituitary system) predispose the patients to multiple clinical manifestations, the severity of which depends on the location, the size and the growth potential of the tumour. Headaches, nausea/vomiting, visual disturbances, growth failure (in children) and hypogonadism (in adults) are the most frequently reported. The hypothalamo-pituitary function at presentation may be severely affected and based on various series, GH deficiency is present in 35–100 % of the evaluated patients, FSH/LH deficiency in 38–91 %, ACTH deficiency in 21–68 %, TSH deficiency in 20–42 % and diabetes insipidus in 6–38 %. Early diagnosis is of major importance.