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少突胶质细胞瘤的分子发病机制

Molecular pathogenesis of oligodendroglial tumors.

作者信息

Jeuken Judith W M, von Deimling Andreas, Wesseling Pieter

机构信息

Department of Pathology, University Medical Centre Nijmegen, Nijmegen, The Netherlands.

出版信息

J Neurooncol. 2004 Nov;70(2):161-81. doi: 10.1007/s11060-004-2748-1.

Abstract

Based on their histopathological appearances, most diffusely infiltrative gliomas can be classified either as astrocytic tumors (As), pure oligodendroglial tumors (Os) or mixed oligoastrocytic tumors (OAs). The latter two may be grouped together as oligodendroglial tumors (OTs). The distinction between As and OTs is important because of the more favorable clinical behavior of OTs. Unfortunately, the histopathological delineation of OAs, Os and As can be difficult because of vague and subjective histopathological criteria. Over the last decade, the knowledge on the molecular genetic background of OTs has drastically increased. This review provides an overview of molecular genetic aberrations in OTs and discusses the pathobiological and clinical significance of these aberrations. In contrast to As, OTs frequently show frequent loss of heterozygosity on chromosome arms 1p and 19q. Since these aberrations are significantly correlated with clinically relevant parameters, such as prognosis and chemosensitivity, and given the difficulties in histopathological typing and grading of glial tumors, genetic testing should be included in routine glioma diagnostics. It is to be expected that the identification of the relevant tumor suppressor genes located on 1p and 19q will lead to more refined genetic tests for OTs. Furthermore, as microarray technology is rapidly increasing, it is likely that clinically relevant markers for OTs will be identified on other chromosomes and need to be included into routine glioma diagnostics as well.

摘要

根据其组织病理学表现,大多数弥漫性浸润性胶质瘤可分为星形细胞瘤(As)、纯少突胶质细胞瘤(Os)或混合性少突星形细胞瘤(OAs)。后两者可归为少突胶质细胞瘤(OTs)。As和OTs的区分很重要,因为OTs的临床行为更有利。不幸的是,由于组织病理学标准模糊且主观,OAs、Os和As的组织病理学鉴别可能很困难。在过去十年中,关于OTs分子遗传背景的知识有了大幅增加。本综述概述了OTs中的分子遗传异常,并讨论了这些异常的病理生物学和临床意义。与As不同,OTs经常在染色体臂1p和19q上出现杂合性缺失。由于这些异常与预后和化疗敏感性等临床相关参数显著相关,且考虑到胶质细胞瘤组织病理学分型和分级存在困难,基因检测应纳入常规胶质瘤诊断。预计位于1p和19q上的相关肿瘤抑制基因的鉴定将导致针对OTs的更精细的基因检测。此外,随着微阵列技术的迅速发展,很可能在其他染色体上鉴定出OTs的临床相关标志物,也需要纳入常规胶质瘤诊断。

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