First Department of Propaedeutic Internal Medicine, National and Kapodistrian University of Athens, Athens, Greece.
WISDEM Centre, University Hospital of Coventry and Warwickshire, Coventry, United Kingdom.
J Clin Endocrinol Metab. 2019 Aug 1;104(8):3108-3123. doi: 10.1210/jc.2018-01871.
Although most sellar lesions are related to pituitary adenomas, the region gives rise to a variety of neoplasms that can be associated with substantial morbidity and/or mortality.
Information from reviews and guidelines of relevant societies dealing with such neoplasms, as well as articles that have provided new developments that made important contributions to their pathogenesis and treatment up to 2018, were obtained: public indexes such as PubMed/MEDLINE were used with the relevant search items.
Sellar neoplasms have a worse outcome than pituitary adenomas that is related not only to their natural history but also to side effects of therapies and evolving endocrine and/or hypothalamic deficiencies. Recent imaging advances have established the radiological fingerprint of some of these neoplasms, and several chromosomal aberrations have also been identified. Although established approaches along with new surgical and radiotherapeutic approaches remain the main treatment modalities, recent evidence has provided insight into their molecular pathogenesis involving, other than chemotherapy, treatments with targeted agents as in gliomas and craniopharyngiomas bearing BRAF mutations. Development of predictive markers of recurrences may also identify high-risk patients, including proliferative markers and expression of the progesterone receptor in meningiomas, and lead to less aggressive surgery. Owing to the rarity and complexity of these neoplasms, patients should be managed in dedicated centers.
The diagnosis and management of sellar neoplasms necessitate a multidisciplinary approach. Following evolving recent advances in their diagnosis and therapy, such a multidisciplinary approach needs to be extended to establish evidence-based diagnostic and management plans.
尽管大多数鞍区病变与垂体腺瘤有关，但该区域也会产生多种肿瘤，这些肿瘤可能会导致相当大的发病率和/或死亡率。
获取了有关处理此类肿瘤的相关学会的综述和指南信息，以及截至 2018 年提供了对其发病机制和治疗具有重要贡献的新进展的文章：使用了 PubMed/MEDLINE 等公共索引，并使用了相关搜索项。
鞍区肿瘤的预后比垂体腺瘤差，这不仅与它们的自然病史有关，还与治疗的副作用以及内分泌和/或下丘脑功能逐渐缺失有关。最近的影像学进展确立了其中一些肿瘤的影像学特征，并且还确定了几种染色体异常。尽管已有的治疗方法以及新的手术和放射治疗方法仍然是主要的治疗方式，但最近的证据提供了对其分子发病机制的深入了解，除了化疗外，针对 BRAF 突变的胶质瘤和颅咽管瘤等还可以使用靶向药物治疗。预测复发的生物标志物的开发也可以识别高危患者，包括脑膜瘤中的增殖标志物和孕激素受体的表达，并导致手术侵袭性降低。由于这些肿瘤罕见且复杂，患者应在专门的中心进行治疗。
鞍区肿瘤的诊断和治疗需要多学科方法。在其诊断和治疗方面不断发展的最新进展之后，这种多学科方法需要扩展，以建立基于证据的诊断和管理计划。
