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中枢神经系统淋巴增殖性疾病神经影像学表现谱

英文原题:Spectrum of Neuroimaging Findings in CNS Lymphoproliferative Disorders.

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Spectrum of Neuroimaging Findings in CNS Lymphoproliferative Disorders.

PubMed 2026/09/10(内容时间) Diagnostics (Basel) Q1 · IF 3.8(JCR 2025)

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中文摘要

中枢神经系统(CNS)淋巴增殖性疾病包括一组异质性罕见病种,在诊断和治疗方面均构成重大挑战。原发性 CNS 大 B 细胞淋巴瘤(LBCL)是最常见的形式,也是仅次于胶质瘤的第二常见原发性 CNS 肿瘤。世界卫生组织造血与淋巴组织肿瘤分类第 5 版引入了更新的术语,包括免疫豁免部位的原发性 LBCL 以及免疫缺陷/免疫失调相关淋巴瘤,以及较少见的变异型,如血管内大 B 细胞淋巴瘤、淋巴瘤样肉芽肿病、大脑淋巴瘤病、继发性 CNS 淋巴瘤、硬脑膜黏膜相关淋巴组织(MALT)淋巴瘤以及 T 细胞/NK 细胞淋巴瘤。

展开英文摘要原文

Central nervous system (CNS) lymphoproliferative disorders comprise a heterogeneous group of rare entities that present major diagnostic and therapeutic challenges. Primary CNS large B-cell lymphoma (LBCL) is the most common form and the second most frequent primary CNS tumor after gliomas. The 5th edition of the World Health Organization Classification of Hematolymphoid Tumours introduced updated terminology, including primary LBCL of immune-privileged sites and lymphomas arising in immunodeficiency/dysregulation, along with less common variants such as intravascular large B-cell lymphoma, lymphomatoid granulomatosis, lymphomatosis cerebri, secondary CNS lymphoma, mucosa-associated lymphoid tissue (MALT) lymphoma of the dura, and T-cell/NK-cell lymphomas.

Immune status, Epstein-Barr virus association, and imaging features may assist differentiation, although biopsy remains essential for definitive diagnosis. On MRI, lesions are typically periventricular or involve the basal ganglia, showing low T2 signal intensity, restricted diffusion, and intense enhancement. Spectroscopy demonstrates elevated choline/N-acetylaspartate ratios with lipids, and perfusion is generally lower than in other neoplasms.

Corticosteroid administration before biopsy should be avoided due to potential cytolysis of tumor cells. Major differential diagnoses include glioblastoma, metastases, demyelinating lesions, and infections such as toxoplasmosis, fungal abscess, and tuberculosis. Treatment may cause neurotoxicity and immunosuppression, predisposing patients to opportunistic infections.

论文信息

作者
Taborda MH、Kleinubing JR Jr、Lauriano DRL、Souza JVO、Kami L、Yared JH、Telles BA、da Silva EB Jr
单位
Health Sciences and Internal Medicine Postgraduate Program, Health Sciences Deparment, Federal University of Paraná (PPGMICS-UFPR), Rua General Carneiro 181, Curitiba 82860-140, Paraná, Brazil.Brazil
文献类型
综述
期刊
Diagnostics (Basel, Switzerland)2026 Sep 10
原文标识
PubMed 42793717 · DOI 10.3390/diagnostics16182932