Ependymomas are primary central nervous system tumors (CNS), arising within the posterior fossa and supratentorial regions of the brain, and in the spine. Over the last decade, research has resulted in substantial insights into the molecular characteristics of ependymomas, and significant advances have been made in the establishment of a molecular classification system. Ependymomas both within and between the three CNS regions in which they arise, have been shown to contain distinct genetic, epigenetic and cytogenic aberrations, with at least three molecularly distinct subgroups identified within each region. However, these advances in molecular characterization have yet to be translated into clinical practice, with the standard treatment for ependymoma patients largely unchanged. This review summarizes the advances made in the molecular characterization of intracranial ependymomas, outlines the progress made in establishing preclinical models and proposes strategies for moving toward subgroup-specific preclinical investigations and treatment.

中文翻译：

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颅内室管膜瘤：分子见解和治疗转化。

室管膜瘤是原发性中枢神经系统肿瘤 (CNS)，起源于大脑的后颅窝和幕上区域，以及脊柱。在过去的十年中，研究已经对室管膜瘤的分子特征有了实质性的了解，并且在建立分子分类系统方面取得了重大进展。室管膜瘤在它们出现的三个 CNS 区域内和之间，已被证明包含不同的遗传、表观遗传和细胞遗传畸变，每个区域内至少有三个分子不同的亚群。然而，分子表征方面的这些进展尚未转化为临床实践，室管膜瘤患者的标准治疗方法基本未变。