Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK; University College London Great Ormond Street Institute of Child Health, London, UK.
King's College Hospital NHS Foundation Trust, London, UK.
Lancet Diabetes Endocrinol. 2023 Sep;11(9):694-706. doi: 10.1016/S2213-8587(23)00162-6. Epub 2023 Aug 4.
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.
尽管罕见,但颅咽管瘤构成了儿童下丘脑-垂体区域肿瘤的 80%。尽管是良性的,但这些肿瘤靠近视觉通路、下丘脑和垂体,这意味着肿瘤的治疗和肿瘤本身都会导致明显的长期神经内分泌发病率,而总体存活率较高。迄今为止,这些肿瘤的最佳治疗策略仍未确定,不同中心的治疗方法存在差异。有鉴于此,作为英国一项制定罕见儿科内分泌肿瘤管理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,并在本综述中介绍。这些指南是在英国儿童癌症和白血病组以及英国儿科内分泌学会的主持下制定的,并得到了皇家儿科学会的监督和认可,使用评估研究和评估 II 方法学为患有颅咽管瘤的儿童和年轻人标准化护理。