Neurosurgery Resident from Hospital Universitário Cajuru, Curitiba, Brazil.
Medical Student from Pontifical Catholic University of Paraná, Curitiba, Brazil.
J Clin Neurosci. 2022 May;99:10-16. doi: 10.1016/j.jocn.2022.02.030. Epub 2022 Feb 25.
Intracranial ependymal cysts (IECs) are rare, histologically benign neuroepithelial cysts that mostly occur in the cerebral parenchyma. The majority of these cysts are clinically silent and discovered incidentally, but when symptomatic they may compress surrounding structures, thus surgical intervention is needed. The current data in the literature about ECs is very scarce, and many are misdiagnosed, once they share many radiological characteristics with a variety of intracranial benign cysts. Also their terminology is confusing, and its definitive diagnosis can only be made through a thorough histopathological study, hence a detailed description about these uncommon lesions is necessary. The correct identification of the lesion lead to our better understanding of the condition and further improvement of the patient's prognosis.
A descriptive case is presented; moreover, a detailed PubMed search according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guideline was performed. The data found was analyzed by various criteria in order to correctly describe the characteristics of this lesion.
The literature review gathered 9 descriptions of patients with IECs with a diverse range anatomopathological and clinical manifestations. All of the included studies found were case reports. Moreover, the authors suggest an updated classification of the lesion, involving their immunohistochemical characteristics.
The information obtained from this study highlights IECs rarity and their inaccurately classification. We propose that the definitive diagnosis of IECs shall be made upon histopathological confirmation of an ependyma-lined cyst along with a positive glial fibrillary acidic protein (GFAP).
颅内室管膜囊肿（IECs）是罕见的组织学良性神经上皮囊肿，主要发生在脑实质。这些囊肿大多数是无症状的偶然发现，但当出现症状时，它们可能会压迫周围结构，因此需要手术干预。目前文献中关于 ECS 的数据非常有限，许多病例被误诊，因为它们与多种颅内良性囊肿有许多相似的影像学特征。此外，它们的术语也令人困惑，只有通过彻底的组织病理学研究才能做出明确的诊断，因此有必要对这些罕见病变进行详细描述。正确识别病变有助于我们更好地了解病情，并进一步改善患者的预后。
呈现一个描述性病例；此外，根据系统评价和荟萃分析的首选报告项目（PRISMA）指南进行了详细的 PubMed 搜索。通过各种标准对收集到的数据进行分析，以正确描述该病变的特征。
文献复习共收集了 9 例 IECs 患者的描述，其解剖病理学和临床表现各不相同。所有纳入的研究均为病例报告。此外，作者建议对病变进行更新的分类，包括其免疫组织化学特征。
从这项研究中获得的信息强调了 IECs 的罕见性及其分类不准确。我们建议，IECs 的明确诊断应基于组织病理学证实存在室管膜衬里的囊肿，并伴有胶质纤维酸性蛋白（GFAP）阳性。
