中枢神经系统淋巴瘤。

IF 1.8 4区 医学 Q3 CLINICAL NEUROLOGY Seminars in Neurology Pub Date : 2023-12-01 Epub Date: 2023-11-23 DOI:10.1055/s-0043-1776783
Trusha Shah, Vyshak A Venur
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引用次数: 0

摘要

中枢神经系统淋巴瘤(CNSL)是一种罕见的侵袭性恶性肿瘤,主要影响大脑、脊髓和脑膜。本文全面概述了目前对CNSL的理解,包括其流行病学、病理生理学、临床表现、诊断、治疗方式和预后。虽然主要的焦点是原发性中枢神经系统淋巴瘤(PCNSL),但眼部淋巴瘤、原发性轻脑膜淋巴瘤和继发性中枢神经系统淋巴瘤也被讨论。CNSL的病理机制包括恶性淋巴细胞在中枢神经系统实质或脑膜内的浸润。各种危险因素和免疫机制有助于其发展,包括免疫缺陷状态,慢性炎症和基因组改变。准确的诊断对于适当的治疗是至关重要的,鉴于异质的临床表现。我们将讨论神经影像学、全身影像学和其他诊断和评估疾病累及程度的方法。此外,强调了组织病理学检查、脑脊液(CSF)分析和分子检测在确诊和指导治疗决策中的重要性。CNSL的治疗前景已经发生了重大变化。治疗方法包括多模式策略,结合高剂量甲氨蝶呤为基础的化疗,巩固全脑放疗,高剂量化疗与干细胞抢救。靶向治疗和免疫调节剂的最新进展为未来的治疗选择提供了有希望的途径。我们回顾了影响CNSL患者生存的临床结果和预后因素,包括年龄、运动状态、疾病分期和遗传异常。
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Central Nervous System Lymphoma.

Central nervous system lymphoma (CNSL) is a rare and aggressive malignancy that primarily affects the brain, spinal cord, and meninges. This article provides a comprehensive overview of the current understanding of CNSL encompassing its epidemiology, pathophysiology, clinical presentation, diagnosis, treatment modalities, and prognosis. Although the main focus is on primary CNS lymphoma (PCNSL), ocular lymphoma, primary leptomeningeal lymphoma, and secondary CNS lymphoma are also discussed. The pathobiology of CNSL involves the infiltration of malignant lymphocytes within the CNS parenchyma or leptomeninges. Various risk factors and immunological mechanisms contribute to its development, including immunodeficiency states, chronic inflammation, and genomic alterations. Accurate diagnosis is crucial for appropriate management, given the heterogeneous clinical presentation. The neuroimaging, systemic imaging, and other modalities for diagnosis and evaluation for extent of disease involvement will be discussed. Additionally, the importance of histopathological examination, cerebrospinal fluid (CSF) analysis, and molecular testing in confirming the diagnosis and guiding treatment decisions are highlighted. The treatment landscape for CNSL has evolved significantly. Therapeutic approaches encompass a multimodal strategy combining high-dose methotrexate-based chemotherapy, consolidation with whole-brain radiation therapy, and high-dose chemotherapy with stem cell rescue. Recent advancements in targeted therapies and immunomodulatory agents offer promising avenues for future treatment options. We review the clinical outcomes and prognostic factors influencing the survival of CNSL patients, including age, performance status, disease stage, and genetic abnormalities.

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来源期刊
Seminars in Neurology
Seminars in Neurology 医学-临床神经学
CiteScore
4.60
自引率
3.70%
发文量
65
审稿时长
6-12 weeks
期刊介绍: Seminars in Neurology is a review journal on current trends in the evaluation, diagnosis, and treatment of neurological diseases. Areas of coverage include multiple sclerosis, central nervous system infections, muscular dystrophy, neuro-immunology, spinal disorders, strokes, epilepsy, motor neuron diseases, movement disorders, higher cortical function, neuro-genetics and neuro-ophthamology. Each issue is presented under the direction of an expert guest editor, and invited contributors focus on a single, high-interest clinical topic. Up-to-the-minute coverage of the latest information in the field makes this journal an invaluable resource for neurologists and residents.
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