Archive | 2021
Childhood-onset craniopharyngiomas
Abstract
Abstract Childhood-onset craniopharyngiomas are rare, partly cystic, and calcified embryonic malformations of the sellar/parasellar region with low histological grade (WHO Io). Clinical manifestations are related to hypothalamic/pituitary deficits, visual impairment, and elevated intracranial pressure. If the tumor is favorably localized, therapy of choice is complete resection, with care taken to preserve optical and hypothalamic functionality. In patients with unfavorable tumor location, recommended therapy is limited hypothalamus-sparing surgical strategy followed by local irradiation. Even though overall survival rates are high (92%), recurrences and progressions are frequent. Irradiation has proven effective in reducing recurrences and progression. Anatomical involvement and/or surgical lesions of posterior hypothalamic areas can result in serious quality of life, compromising sequelae such as hypothalamic obesity and neuropsychological sequelae. It is crucial that childhood-onset craniopharyngioma be managed as a frequently chronic disease, providing ongoing care of pediatric and adult patients’ clinical and quality of life consequences by experienced multidisciplinary teams.