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The craniopharyngioma is a rare dysontogenetic tumour that originates from either scattered cells of the craniopharyngeal duct or from metaplastically mutated anterior pituitary parenchyma cells. Despite being classified as a WHO-Class-I tumour, the histologically benign craniopharyngioma may display an aggressive behaviour. Like other congenital tumours, it usually becomes manifest within the first two decades of life. Patients typically complain of headache and a chiasma syndrome with bitemporal hemianopsy may develop depending on tumour localisation. In children, anterior pituitary insufficiency often manifests as growth restriction. Additionally, diabetes insipidus and other hormonal disturbances may develop. Therapeutically either radical total removal or subtotal resection in combination with postoperative radiation is recommended. In cystic tumors, stereotactic cyst drainage and adjuvant radiation may be a possible alternative. The prognosis is best in patients who are diagnosed early.