鞍旁区域是蝶鞍周围的解剖区域，代表重要相邻结构的关键十字路口。几种不同的肿瘤主要起源于该区域，最常见的是脑膜瘤、神经胶质瘤、胚胎细胞肿瘤、生殖细胞肿瘤和颅咽管瘤。此外，许多全身性疾病和炎症性疾病也会影响鞍旁区，最常见的是垂体。根据新的 WHO CNS5 2021 分类，这些病变具有不同的病理特征和恶性潜力。体征和症状可能是非特异性的，主要与周围解剖结构的占位效应和/或内分泌功能受损有关，而绝大多数缺乏分泌成分。基于分子技术进步的突变特征分析最近已经能够识别特定的基因突变或信号通路畸变。这些进展可能成为描述这些病变的病理生理学的有力手段，并作为诊断、预后和治疗工具，特别是对于高危人群。治疗选择包括单独手术或与放疗、化疗和特定疾病的药物治疗相结合，以防止肿瘤复发或进一步生长，同时替代共存的垂体激素缺乏。在这篇全面的综述中，我们介绍了这些病变的组织病理学和分子生物学的最新进展，这些进展可由相关专业的专门多学科团队用于诊断、监测和治疗鞍旁病变，这些病变通常代表了诊断和治疗的挑战。© 作者 2024。由牛津大学出版社代表内分泌学会出版。版权所有。如需商业重复使用，请联系 reprints@oup.com 获取转载和转载的翻译权。所有其他权限都可以通过我们网站文章页面上的权限链接通过我们的 RightsLink 服务获得 - 如需了解更多信息，请联系journals.permissions@oup.com。有关附加条款，请参阅期刊“关于”页面。

The parasellar region is the anatomical area around the sella turcica that represents a crucial crossroad for important adjacent structures. Several distinct tumors can primarily originate from this area, the most common being meningiomas, gliomas, embryonal cell tumors, germ cell tumors and craniopharyngiomas. In addition, a number of systemic and inflammatory disorders can also affect the parasellar region most commonly involving the pituitary. These lesions have different pathology characteristics and malignant potential according to the new WHO CNS5 2021 classification. Signs and symptoms may be non-specific and are mostly related to a mass effect on the surrounding anatomical structures and/or impairment of endocrine function whereas the vast majority lack a secretory component. The mutational signature analysis based on advances in molecular techniques, has recently enabled the identification of specific gene mutations or signalling pathway aberrations. These developments may serve as a powerful mean to delineate the pathophysiology of these lesions and serve as a diagnostic, prognostic and therapeutic tool, particularly for high-risk populations. Treatment options include surgery alone or in combination with radiotherapy, chemotherapy and disease-specific medical therapy in order to prevent recurrence or further tumor growth along with replacement of coexistent pituitary hormonal deficiencies. In this comprehensive review, we present current state-of-the-art developments in the histopathology and molecular biology of these lesions that may be utilized by a dedicated multidisciplinary team of relevant specialties for the diagnosis, monitoring and treatment of the parasellar lesions that often represent a diagnostic and therapeutic challenge.© The Author(s) 2024. Published by Oxford University Press on behalf of the Endocrine Society. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact journals.permissions@oup.com. See the journal About page for additional terms.