Cachia David, Johnson Derek R, Kaufmann Timothy J, Lowe Stephen, Andersen Samuel, Olar Adriana, Cooper Samuel Lewis, Frankel Bruce M, Gilbert Mark R
Department of Neuro-surgery, Medical University of South Carolina, Charleston, USA.
Department of Neurology and Division of Medical Oncology, Mayo Clinic, Rochester, Minnesota, USA.
Neurooncol Pract. 2018 Aug;5(3):142-153. doi: 10.1093/nop/npy026. Epub 2018 Jul 24.
Ependymomas are rare primary central nervous system (CNS) tumors in adults. They occur most commonly in the spinal cord, and have classically been graded histologically into World Health Organization (WHO) grades I, II, or III based on the level of anaplasia. Recent data are showing that genetic heterogeneity occurs within the same histological subgroup and that ependymomas arising from different CNS locations have different molecular signatures. This has renewed interest in developing targeting therapies based on molecular profiles especially given the variable outcomes with radiation and the poor results with cytotoxic agents. In this paper, we present the case of a 46-year-old woman with a classic presentation of spinal cord ependymoma and discuss the current histopathological and molecular classification for ependymomas as well as current guidelines for patient management.
室管膜瘤是成人中罕见的原发性中枢神经系统（CNS）肿瘤。它们最常发生于脊髓，传统上根据间变程度在组织学上被分为世界卫生组织（WHO）I级、II级或III级。最近的数据表明，在同一组织学亚组内存在基因异质性，并且起源于不同CNS部位的室管膜瘤具有不同的分子特征。鉴于放疗效果不一且细胞毒性药物疗效不佳，这重新激发了人们基于分子特征开发靶向治疗的兴趣。在本文中，我们报告了一名46岁女性脊髓室管膜瘤的典型病例，并讨论了目前室管膜瘤的组织病理学和分子分类以及患者管理的现行指南。
