Clinicopathologic and genetic profile of intracranial marginal zone lymphoma: A primary low-grade CNS lymphoma that mimics meningioma
Clinicopathologic and genetic profile of intracranial marginal zone lymphoma: A primary low-grade CNS lymphoma that mimics meningioma
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DOI：
10.1200/jco.2005.17.624
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发表时间：
2005-08-20
影响因子：
45.3
通讯作者：
Perry, A
中科院分区：
文献类型：
--
作者：
Tu, PH;Giannini, C;Perry, A
目的 虽然边缘区 B 细胞淋巴瘤 (MZBCL) 总体罕见，但它是文献报道的最常见的原发性低级别 CNS 淋巴瘤。本研究的目的是阐明这种罕见肿瘤的生物学和遗传特征。患者和方法对 15 例 CNS MZBCL 进行了临床、病理和遗传学研究，包括使用市售的 MALT1 和 IgH 断裂以及着丝粒 3、7、12 和 18 探针进行荧光原位杂交分析。结果 CNS MZBCL 优先影响中年女性（男女比例为 4:1），其中 93% 表现为类似脑膜瘤的硬脑膜肿块。诊断后 1 至 7.6 年的随访中，10 名患者在放疗和/或化疗后未发现疾病证据。与 CNS 之外的 MZBCL 一样，它们由 CD20(+)、CD3(-) 小 B 淋巴细胞组成，具有不同程度的浆细胞分化，并且主要受 K 轻链限制 (78%)。在三名患者中观察到具有滤泡定植的淋巴滤泡，在两名患者的样本中发现淀粉样蛋白沉积，其中一名患者出现肿胀。 Bcl-6 蛋白和 Epstein-Barr 病毒编码的 RNA 均未表达。 12 名患者中有 6 名检测到 3 三体性，未检测到 MALT1 或 IgH 重排，也未检测到 7、12 或 18 三体性。 结论 我们的数据表明颅内 MZBCL 是一种惰性原发性 CNS 淋巴瘤，通常表现为脑膜瘤样硬脑膜肿块。 3 三体，而非 MALT1 或 IgH 易位，是一种常见的遗传异常，可能导致这种中枢神经系统淋巴瘤的发病机制。
Purpose Although rare overall, marginal zone B-cell lymphoma (MZBCL) is the most common primary low-grade CNS lymphoma reported in the literature. The aim of this study is to elucidate the biology and genetic features of this unusual tumor.Patients and Methods Fifteen CNS MZBCLs were studied clinically, pathologically, and genetically, including fluorescent in situ hybridization analyses with commercially available MALT1 and IgH break-apart and centromere 3, 7, 12, and 18 probes.Results CNS MZBCLs preferentially affect middle-aged women (female-to-male ratio, 4:1), with 93% presenting as dural-based masses mimicking meningioma. Ten patients with 1 to 7.6 years of follow-up after diagnosis showed no evidence of disease after radiation and/or chemotherapy. Like MZBCLs outside of the CNS, they consisted of CD20(+), CD3(-) small B lymphocytes with varying degrees of plasmacytic differentiation and predominantly K light-chain restriction (78%). Lymphoid follicles with follicular colonization were seen in three patients and deposition of amyloid was noted in samples from two patients, one of which was tumefactive. Neither Bcl-6 protein nor Epstein-Barr virus-encoded RNA was expressed. Trisomy 3 was detected in six of 12 patients, with no rearrangements of MALT1 or IgH and no trisomies of 7, 12, or 18 detected.Conclusion Our data suggest that intracranial MZBCL is an indolent primary CNS lymphoma that typically presents as a meningioma-like dural-based mass. Trisomy 3, but not MALT1 or IgH translocation, is a common genetic abnormality that may contribute to the pathogenesis of this CNS lymphoma.