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 pathologic characteristics and malignant potential according to the new World Health Organization CNS5 2021 classification. Signs and symptoms may be nonspecific 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 signaling pathway aberrations. These developments may serve as a powerful means 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 the current state-of-the-art developments in the histopathology and molecular biology of parasellar lesions, which often represent a diagnostic and therapeutic challenge, that may be utilized by a dedicated multidisciplinary team for the diagnosis, monitoring, and treatment of these lesions.

中文翻译：

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鞍旁瘤的分子发育和潜在的治疗意义。


鞍旁区域是蝶鞍周围的解剖区域，代表了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、胚胎细胞瘤、生殖细胞瘤和颅咽管瘤。此外，许多全身性疾病和炎症性疾病也会影响鞍旁区域，最常见的是累及垂体。根据新的世界卫生组织 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能够识别特定基因突变或信号通路畸变。这些发展可以作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联合放疗、化疗和疾病特异性药物治疗，以防止复发或肿瘤进一步生长，同时替代并存的垂体激素缺乏症。在这篇全面的综述中，我们介绍了鞍旁病变组织病理学和分子生物学的当前最新进展，这通常代表诊断和治疗挑战，可由专门的多学科团队用于诊断、监测和治疗这些病变。