Molecular pathogenesis of primary central nervous system lymphoma.
Molecular pathogenesis of primary central nervous system lymphoma.
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原发性中枢神经系统淋巴瘤的分子发病机制。
DOI：
10.3171/foc.2006.21.5.2
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发表时间：
2006
影响因子：
4.1
通讯作者：
J. Rubenstein
中科院分区：
文献类型：
--
作者：
Cigall Kadoch;P. Treseler;J. Rubenstein
原发性中枢神经系统淋巴瘤(PCNSL)是一种侵袭性的非霍奇金淋巴瘤(NHL)，与其他具有相似组织学特征的局限性结外淋巴瘤相比，预后较差。PCNSL的主要特征是局限于中枢神经系统(CNS)，有在软脑膜和眼内室内生长的倾向。原发中枢神经系统淋巴瘤很少扩散到中枢神经系统以外，在所有原发脑肿瘤中所占比例不到5%。至少95%的PCNSL是大B细胞组织学，这是NHL最常见的亚型。与全身性NHL的趋势一致，PCNSL的发病率在过去30年中显著增加，无论是在免疫低下的患者还是免疫功能正常的患者中。由于PCNSL相对罕见，分子预后生物标志物的识别和标准治疗策略的定义一直是具有挑战性的。作者讨论了目前对中枢神经系统淋巴瘤分子发病机制的认识，并综述了基因表达谱分析和识别新的预后生物标志物的最新进展。
Primary central nervous system lymphoma (PCNSL) is an aggressive form of non-Hodgkin lymphoma (NHL) typically associated with a worse prognosis than other localized extranodal lymphomas with similar histological characteristics. The defining feature of PCNSL is its confinement to the central nervous system (CNS), with proclivity for growth within the leptomeningeal as well as intraocular compartments. Primary CNS lymphoma rarely disseminates outside the CNS and accounts for less than 5% of all primary brain neoplasms. At least 95% of PCNSLs are of large B-cell histology, the most common subtype of NHL. Consistent with the trend seen in systemic NHLs, the incidence of PCNSL has markedly increased over the past three decades, both in immunocompromised and immunocompetent patients. Because PCNSL is relatively rare, the identification of molecular prognostic biomarkers and the definition of a standard therapeutic strategy have been challenging. The authors discuss the current knowledge of the molecular pathogenesis of CNS lymphomas and review the recent advances in gene expression profile analysis and identification of novel prognostic biomarkers.