Craniopharyngiomas are partly cystic embryogenic malformations of the sellar and parasellar region. With an overall incidence of 0.5-2.0 new cases per million population per year, approximately 30-50% of all cases represent childhood craniopharyngioma. Typical manifestations at diagnosis are some combination of headache, visual impairment, polyuria/polydypsia, growth retardation, and significant weight gain. Therapy of choice in patients with favorable tumor localization is complete resection with specific focus on maintaining functions of the optic nerve and hypothalamic-pituitary axes. In patients whose unfavorable tumor localization makes maintaining hypothalamic functionality surgically challenging, a limited resection followed by local irradiation is recommended. The overall survival rates are high (92%) but occurrences of reduced quality of life are also high. Recurrences after complete resection and progressions of residual tumor after incomplete resection are frequent postsurgical events. Because irradiation is efficient in preventing tumor progression, appropriate timing of postsurgical irradiation is currently under investigation in the randomized multinational trial KRANIOPHARYNGEOM 2007 that analyzes quality of life as primary endpoint. Childhood craniopharyngioma should be recognized as a chronic disease requiring constant monitoring of the clinical and quality of life consequences in order to provide optimal care of surviving patients.
Keywords: Craniopharyngioma; brain tumors; hypothalamic obesity; irradiation; neurocognitive; neurosurgery; pituitary; quality of life; recurrence; sleep.
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