Since the 1990s, the sheer number of defined central nervous system (CNS) embryonal tumor entities has continuously increased, with the trend accelerating in the most recent editions of the World Health Organization (WHO) Classification of Tumours of the CNS. The introduction of increasingly specific tumor groups is an effort to create more internally homogeneous categories, to allow more precise prognostication, and potentially to develop targeted therapies. However, these ever-smaller categories within an already rare group of tumors pose a challenge for pediatric pathologists. In this article we review the current categorization of non-medulloblastoma CNS embryonal tumors (including atypical teratoid/rhabdoid tumor, cribriform neuroepithelial tumor, embryonal tumor with multilayered rosettes, CNS neuroblastoma, FOXR2-activated, and CNS tumor with BCOR internal tandem duplication) and provide an overview of available ancillary techniques to characterize these tumors. We provide a practical approach to workup and development of an integrated diagnosis for CNS embryonal tumors.

中文翻译：

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中枢神经系统胚胎肿瘤（非髓母细胞瘤）的评估和诊断。

自 1990 年代以来，确定的中枢神经系统 (CNS) 胚胎肿瘤实体的绝对数量不断增加，在最新版本的世界卫生组织 (WHO) 中枢神经系统肿瘤分类中，这一趋势正在加速。引入越来越具体的肿瘤组是为了创建更多内部同质的类别，以实现更精确的预后，并有可能开发靶向治疗。然而，在已经非常罕见的一组肿瘤中，这些越来越小的类别对儿科病理学家构成了挑战。在本文中，我们回顾了当前非髓母细胞瘤 CNS 胚胎肿瘤的分类（包括非典型畸胎样/横纹肌样肿瘤、筛状神经上皮肿瘤、具有多层玫瑰花结的胚胎肿瘤、CNS 神经母细胞瘤、FOXR2 激活、和具有 BCOR 内部串联重复的 CNS 肿瘤），并概述了描述这些肿瘤的可用辅助技术。我们提供了一种实用的方法来检查和开发 CNS 胚胎肿瘤的综合诊断。