Ependymes。

IF 4.4 Q1 PATHOLOGY
PATHOLOGICA Pub Date : 2022-12-01 DOI:10.32074/1591-951X-817
Luca Bertero, Alessia Andrea Ricci, Cristian Tampieri, Paola Cassoni, Piergiorgio Modena
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引用次数: 4

摘要

室管膜肿瘤是一种异质性肿瘤,起源于脑室系统和脊髓中央管内衬细胞的祖细胞。在过去的几年中,收集了有关肿瘤发生、分子特征和临床相关性的重要新数据,并与它们的病理分类有很强的相关性。最近出版的《世卫组织中枢神经系统肿瘤分类》第五版整合了这一新知识,与前一版相比有了重大更新。关于膜上室管膜瘤,先前的RELA融合阳性室管膜瘤已更名为ZFTA融合阳性,并增加了新的YAP1融合阳性室管膜瘤亚型。后窝室管膜瘤现在应该根据分子谱或使用H3 K27me3免疫组织化学替代品来划分为A型或B型亚型。关于脊髓室管膜瘤,基于一种独特的分子特征、MYCN扩增的存在和不利的结果,增加了一种新的亚型。最后,黏液乳头状室管膜瘤根据其总体预后与传统的脊髓室管膜瘤相似,现在被归类为2级肿瘤。本综述的目的是介绍这些变化,并根据最新的更新总结当前室管膜肿瘤的诊断框架。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

Ependymomas.

Ependymomas.

Ependymomas.

Ependymomas.

Ependymal neoplasms are a heterogenous group of neoplasms arising from the progenitors of the cells lining the ventricular system and the spinal central canal. During the last few years, significant novel data concerning oncogenesis, molecular characteristics and clinical correlations of these tumours have been collected, with a strong relevance for their pathological classification. The recently published 5th edition of WHO Classification of Central Nervous System Tumours integrates this novel knowledge and represents a substantial update compared to the previous edition. Concerning supratentorial ependymomas, the previous RELA fusion-positive ependymoma has been renamed into ZFTA fusion-positive and the novel YAP1 fusion-positive ependymoma subtype has been added. Posterior fossa ependymomas should now be allocated either to the Type A or Type B subtypes based on molecular profiling or using the H3 K27me3 immunohistochemical surrogate. Regarding spinal ependymomas, a novel subtype has been added based on a distinctive molecular trait, presence of MYCN amplification, and on the unfavourable outcome. Finally, myxopapillary ependymoma is now classified as a grade 2 tumour in accordance with its overall prognosis which mirrors that of conventional spinal ependymomas. The aim of this review is to present these changes and summarize the current diagnostic framework of ependymal tumours, according to the most recent updates.

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来源期刊
PATHOLOGICA
PATHOLOGICA PATHOLOGY-
CiteScore
5.90
自引率
5.70%
发文量
108
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