Mesh : Adult Male Female Humans Adolescent Child Aged Pituitary Neoplasms / diagnosis therapy pathology Adenoma / diagnosis therapy Acromegaly Prolactinoma / diagnosis surgery

来  源:   DOI:10.1038/s41574-023-00949-7

Abstract:
Pituitary adenomas are rare in children and young people under the age of 19 (hereafter referred to as CYP) but they pose some different diagnostic and management challenges in this age group than in adults. These rare neoplasms can disrupt maturational, visual, intellectual and developmental processes and, in CYP, they tend to have more occult presentation, aggressive behaviour and are more likely to have a genetic basis than in adults. Through standardized AGREE II methodology, literature review and Delphi consensus, a multidisciplinary expert group developed 74 pragmatic management recommendations aimed at optimizing care for CYP in the first-ever comprehensive consensus guideline to cover the care of CYP with pituitary adenoma. Part 2 of this consensus guideline details 57 recommendations for paediatric patients with prolactinomas, Cushing disease, growth hormone excess causing gigantism and acromegaly, clinically non-functioning adenomas, and the rare TSHomas. Compared with adult patients with pituitary adenomas, we highlight that, in the CYP group, there is a greater proportion of functioning tumours, including macroprolactinomas, greater likelihood of underlying genetic disease, more corticotrophinomas in boys aged under 10 years than in girls and difficulty of peri-pubertal diagnosis of growth hormone excess. Collaboration with pituitary specialists caring for adult patients, as part of commissioned and centralized multidisciplinary teams, is key for optimizing management, transition and lifelong care and facilitates the collection of health-related quality of survival outcomes of novel medical, surgical and radiotherapeutic treatments, which are currently largely missing.
摘要:
垂体腺瘤在19岁以下的儿童和年轻人中很少见(以下称为CYP),但与成年人相比,它们在该年龄段的诊断和管理方面存在一些不同的挑战。这些罕见的肿瘤可以破坏成熟,视觉,智力和发展过程,在CYP,他们倾向于有更多的隐秘性表现,攻击性行为,比成年人更有可能有遗传基础。通过标准化的AGREEII方法,文献综述和德尔菲共识,一个多学科专家组制定了74项务实的管理建议,旨在优化CYP的治疗,这是首个全面共识指南,涵盖CYP伴垂体腺瘤的治疗.该共识指南的第2部分详细介绍了针对儿科泌乳素瘤患者的57条建议,库欣病,生长激素过量导致巨人症和肢端肥大症,临床无功能腺瘤,还有罕见的TSHOMA.与成人垂体腺瘤患者相比,我们强调,在CYP组中,有更大比例的功能性肿瘤,包括大型泌乳素瘤,潜在遗传疾病的可能性更大,10岁以下男孩的促肾上腺皮质激素瘤多于女孩,并且难以诊断生长激素过量。与照顾成年患者的垂体专家合作,作为委托和集中的多学科团队的一部分,是优化管理的关键,过渡和终身护理,并促进收集与健康相关的生存质量,手术和放射治疗,目前大部分缺失。
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