Hübner Jens-Martin, Kool Marcel, Pfister Stefan M, Pajtler Kristian W
Division of Pediatric Neurooncology, German Consortium for Translational Cancer Research (DKTK), German Cancer Research Center (DKFZ), Heidelberg, Germany.
Department of Pediatric Hematology and Oncology, Heidelberg University Hospital, Heidelberg, Germany.
J Neurosurg Sci. 2018 Feb;62(1):46-50. doi: 10.23736/S0390-5616.17.04152-2. Epub 2017 Sep 8.
Ependymoma can arise throughout all compartments of the central nervous system with prevalence for intracranial and spinal location in children and adults, respectively. The current histopathology based WHO grading system distinguishes grade I, II 'classic', and III 'anaplastic' ependymoma. However, analysis of multiple cohorts of intracranial ependymoma demonstrate a wide variance in the utility of the grade II versus grade III distinction as a prognostic marker that may additionally be confounded by the anatomic compartment. Recent (epi)genomic profiling efforts have identified molecularly distinct groups of ependymoma arising from all three anatomic compartments of the central nervous system that outperform the current histopathological classification regarding clinical associations. These advances have led to the cognition that molecular classification should be part of all future clinical trials in ependymoma patients. Clinical management of intracranial ependymomas (WHO Grade II/III) is challenging and molecular classification based risk stratification may help to intensify treatment and surveillance in high-risk patients but to de-escalate therapy in certain patient groups at low risk for recurrence. Finally, experience of neurosurgeons, and other disciplines, as well as intensified co-operation between all stakeholders involved hold promise to finally improve outcome of patients affected with ependymoma.
室管膜瘤可发生于中枢神经系统的所有部位，在儿童中颅内室管膜瘤更为常见，在成人中脊髓室管膜瘤更为常见。目前基于组织病理学的世界卫生组织（WHO）分级系统将室管膜瘤分为I级、II级“经典型”和III级“间变型”。然而，对多组颅内室管膜瘤的分析表明，II级与III级区分作为预后标志物的效用存在很大差异，而且可能还会受到解剖部位的影响。最近的（表观）基因组分析研究已经确定了源自中枢神经系统所有三个解剖部位的分子特征不同的室管膜瘤组，在临床相关性方面优于目前的组织病理学分类。这些进展使得人们认识到分子分类应成为未来所有室管膜瘤患者临床试验的一部分。颅内室管膜瘤（WHO II/III级）的临床管理具有挑战性，基于分子分类的风险分层可能有助于加强对高危患者的治疗和监测，但对于某些复发风险低的患者群体则可降低治疗强度。最后，神经外科医生和其他学科的经验，以及所有相关利益者之间加强合作，有望最终改善室管膜瘤患者的治疗效果。
