Institute of Pathology, School of Medicine, University of Belgrade, Belgrade, Serbia.
Section of Neurosurgery, Department of Neuroscience, Uppsala University, Uppsala, Sweden.
Neuroendocrinology. 2020;110(9-10):740-752. doi: 10.1159/000507084. Epub 2020 Mar 11.
The anatomical and histological complexity of the parasellar region as well as the presence of embryonic remnants determine the huge diversity of parasellar neoplasms. Some of them are only located in the parasellar region, whereas others can occur elsewhere, within or outside the central nervous system. Their spectrum ranges from histologically benign and low-grade malignant to high-grade malignant tumours. Although rare, metastases can pose differential diagnostic dilemmas. The severity of the clinical picture, the challenges of surgery and the risk of adverse sequelae related to surgery or radiotherapy make parasellar tumours interesting entities for the clinicians irrespective of their histological malignancy grade. Due to the different cell origins of parasellar tumours, the World Health Organization classification system does not categorise them as a distinct group. Detailed criteria for classification and malignancy grading are presented in the classification systems covering central nervous system tumours, haematological malignancies and tumours of the soft tissue and bone. In the last few years, molecular genetic features have been integrated into the diagnosis of several types of the parasellar tumours enhancing diagnostic accuracy and providing information of the value for targeting therapies. In this review, we will present histopathological and molecular genetic features, updated classification criteria and recent advances in the diagnostics and rationale for novel pharmacological therapies of selected types of parasellar neoplasms.
鞍旁区域的解剖学和组织学复杂性以及胚胎残留物的存在决定了鞍旁肿瘤的巨大多样性。其中一些仅位于鞍旁区域，而另一些则可能位于中枢神经系统内或外部的其他部位。它们的范围从组织学上良性和低度恶性到高度恶性肿瘤。尽管罕见，但转移瘤可能会造成鉴别诊断上的困境。临床症状的严重程度、手术的挑战以及与手术或放疗相关的不良后果风险，使得鞍旁肿瘤成为临床医生感兴趣的实体，无论其组织学恶性程度如何。由于鞍旁肿瘤的不同细胞起源，世界卫生组织分类系统并未将它们归为一个单独的组别。中枢神经系统肿瘤、血液系统恶性肿瘤以及软组织和骨肿瘤的分类系统中详细列出了分类和恶性分级的标准。在过去的几年中，分子遗传学特征已被纳入几种鞍旁肿瘤的诊断中，提高了诊断的准确性，并提供了靶向治疗有价值的信息。在这篇综述中，我们将介绍鞍旁肿瘤的组织病理学和分子遗传学特征、更新的分类标准以及在选定类型的鞍旁肿瘤的诊断和新型药理学治疗方法的最新进展。
