Department of Pediatric Endocrinology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, The Netherlands.
Department of Neuro-Oncology, Princess Maxima Center for Pediatric Oncology, Utrecht, The Netherlands.
Front Horm Res. 2021;54:47-57. doi: 10.1159/000515318. Epub 2021 May 7.
Although childhood-onset craniopharyngioma is a low-grade intracranial tumor with excellent prognosis in terms of overall survival, survivors may suffer from devastating consequences caused by hypothalamic damage. Disease- or treatment-related hypothalamic damage leads to disturbed hunger-satiety and thirst feelings, decreased energy expenditure, behavioral problems, disturbances of circadian rhythm, temperature dysregulation, and pituitary dysfunction. These children are at great risk for developing the metabolic syndrome and comorbidities leading to premature mortality. In this chapter, we shall discuss hypothalamic-pituitary morbidity and outcome of childhood-onset craniopharyngioma patients and future perspectives for improvement.
尽管儿童期颅咽管瘤是一种低级别颅内肿瘤,从总体生存率来看预后良好,但幸存者可能会遭受由于下丘脑损伤引起的毁灭性后果。疾病或治疗相关的下丘脑损伤导致饥饿-饱腹感和口渴感紊乱、能量消耗减少、行为问题、昼夜节律紊乱、体温调节障碍和垂体功能障碍。这些儿童发生代谢综合征和导致过早死亡的合并症的风险很大。在本章中,我们将讨论儿童期颅咽管瘤患者的下丘脑-垂体发病率和结果,以及未来改善的前景。