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首页> 外文期刊>Neuropathology: official journal of the Japanese Society of Neuropathology >Primary central nervous system lymphoma: Immunohistochemical profile and prognostic significance.
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Primary central nervous system lymphoma: Immunohistochemical profile and prognostic significance.

机译：原发性中枢神经系统淋巴瘤：免疫组化谱和预后意义。

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摘要

Primary central nervous system lymphoma (PCNSL) is a rare subtype of non-Hodgkin lymphoma (NHL) with extranodal location affecting only the CNS, meninges and eye, without visceral or lymph node involvement. Its incidence has increased sharply over the past three decades, especially in immunocompetent subjects. Most PCNSL cases are diffuse large B-cell lymphomas (DLBCLs). However, it differs from nodal DLBCL in that it has a worse prognosis. DLBCLs are a heterogeneous entity and according to new genomic discoveries, classifications into prognostic subgroups have been embarked upon. Two prognostic algorithms were then prepared using a panel of immunohistochemical markers (CD10, Bcl6, MUM1/IRF-4, and Bcl2), thus categorizing DLBCL into two subgroups, GCB (germinal centre B-cell-like) or non-GCB, and into Group 1 or Group 2. Our goal is to apply both of these two sub-classifications to 39 PCNSLs, in order to assess their usefulness and prognostic relevance. 74.3% of our PCNSLs were of a non-GCB phenotype, corresponding to an activated postgerminal origin. They were evenly distributed across G1 and G2. Two- and 5-year overall survival rates were 34.8% and 19.6%, respectively. Younger age (<65) and a therapeutic combination of chemotherapy and radiotherapy significantly improved our patients' survival rates. The other clinical or biological markers tested had no prognostic impact. The two classifications did not reveal any significant survival difference. The recent discovery of a specific "transcriptional signature" of PCNSL, marking them out of DLBCL could account for the irrelevance of such prognostic classifications to PCNSL.
机译：原发性中枢神经系统淋巴瘤（PCNSL）是非霍奇金淋巴瘤（NHL）的一种罕见亚型，其结外位置仅影响CNS，脑膜和眼睛，不涉及内脏或淋巴结。在过去的三十年中，其发病率急剧上升，尤其是在具有免疫能力的受试者中。大多数PCNSL病例是弥漫性大B细胞淋巴瘤（DLBCL）。但是，它与结节性DLBCL的不同之处在于预后较差。 DLBCL是一个异质实体，根据新的基因组发现，已开始将其分类为预后亚组。然后使用一组免疫组织化学标记物（CD10，Bcl6，MUM1 / IRF-4和Bcl2）准备了两种预后算法，从而将DLBCL分为两个亚组，即GCB（生发中心B细胞样）或非GCB，以及分为第1组或第2组。我们的目标是将这两个子分类都应用于39个PCNSL，以评估其有用性和预后相关性。我们的PCNSL中74.3％是非GCB表型，对应于激活的发芽后起源。它们平均分布在G1和G2中。两年和五年的总生存率分别为34.8％和19.6％。年龄较小（<65岁）以及化学疗法和放射疗法的治疗组合显着提高了我们患者的生存率。测试的其他临床或生物学标志物对预后没有影响。两种分类均未显示任何明显的生存差异。 PCNSL的特定“转录签名”的最新发现，将其标记为DLBCL，可以解释这种预后分类与PCNSL无关。

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