Abstract：
Although rare, craniopharyngiomas constitute up to 80% of tumours in the hypothalamic-pituitary region in childhood. Despite being benign, the close proximity of these tumours to the visual pathways, hypothalamus, and pituitary gland means that both treatment of the tumour and the tumour itself can cause pronounced long-term neuroendocrine morbidity against a background of high overall survival. To date, the optimal management strategy for these tumours remains undefined, with practice varying between centres. In light of these discrepancies, as part of a national endeavour to create evidence-based and consensus-based guidance for the management of rare paediatric endocrine tumours in the UK, we aimed to develop guidelines, which are presented in this Review. These guidelines were developed under the auspices of the UK Children\'s Cancer and Leukaemia Group and the British Society for Paediatric Endocrinology and Diabetes, with the oversight and endorsement of the Royal College of Paediatrics and Child Health using Appraisal of Guidelines for Research & Evaluation II methodology to standardise care for children and young people with craniopharyngiomas.
摘要：
虽然罕见，颅咽管瘤占儿童期下丘脑-垂体区肿瘤的80％。尽管是良性的，这些肿瘤非常接近视觉通路，下丘脑,和垂体腺意味着在高总生存率的背景下，肿瘤的治疗和肿瘤本身都可以导致明显的长期神经内分泌发病率。迄今为止,这些肿瘤的最佳管理策略仍然不确定，各中心之间的实践各不相同。鉴于这些差异，作为国家努力为英国罕见儿科内分泌肿瘤的管理创建基于证据和基于共识的指导的一部分，我们的目标是制定指导方针，这些都在这篇评论中介绍。这些指南是在英国儿童癌症和白血病小组和英国儿科内分泌和糖尿病学会的主持下制定的，在皇家儿科和儿童健康学院的监督和认可下，使用《研究与评估指南II》方法来标准化颅咽管瘤儿童和年轻人的护理。
