尽管罕见，但颅咽管瘤在儿童的下丘脑-脑垂体区域中占肿瘤的80％。尽管是良性的，但这些肿瘤与视觉通路、下丘脑和脑垂体的密切接触，意味着肿瘤的治疗和肿瘤本身都可能在高总体生存率的背景下引起明显的长期神经内分泌发病率。迄今为止，对于这些肿瘤的最佳管理策略尚未确定，实践在不同中心之间存在差异。鉴于这些差异，作为英国针对罕见儿科内分泌肿瘤制定循证和共识指南的全国性努力的一部分，我们旨在制定指南，并在本综述中予以呈现。这些指南是在英国儿童肿瘤和白血病组织以及英国儿科内分泌学和糖尿病学会的支持和认可下，由皇家儿科和儿童健康学院的评估指南和研究II方法制定的，旨在标准化对患有颅咽管瘤的儿童和年轻人的护理。版权所有© 2023 Elsevier Ltd. 保留所有权利。

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.Copyright © 2023 Elsevier Ltd. All rights reserved.