Department of Neurology, University of California-San Francisco, 505 Parnassus Ave, San Francisco, CA 94143, USA.
JAMA Neurol. 2013 Mar 1;70(3):311-9. doi: 10.1001/jamaneurol.2013.606.
Central nervous system (CNS) lymphoma can present a diagnostic challenge. Currently, there is no consensus regarding what presurgical evaluation is warranted or how to proceed when lesions are not surgically accessible. We conducted a review of the literature on CNS lymphoma diagnosis (1966 to October 2011) to determine whether a common diagnostic algorithm can be generated. We extracted data regarding the usefulness of brain and body imaging, serum and cerebrospinal fluid (CSF) studies, ophthalmologic examination, and tissue biopsy in the diagnosis of CNS lymphoma. Contrast enhancement on imaging is highly sensitive at the time of diagnosis: 98.9% in immunocompetent lymphoma and 96.1% in human immunodeficiency virus-related CNS lymphoma. The sensitivity of CSF cytology is low (2%-32%) but increases when combined with flow cytometry. Cerebrospinal fluid lactate dehydrogenase isozyme 5, β2-microglobulin, and immunoglobulin heavy chain rearrangement studies have improved sensitivity over CSF cytology (58%-85%) but have only moderate specificity (85%). New techniques of proteomics and microRNA analysis have more than 95% specificity in the diagnosis of CNS lymphoma. Positive CSF cytology, vitreous biopsy, or brain/leptomeningeal biopsy remain the current standard for diagnosis. A combined stepwise systematic approach outlined here may facilitate an expeditious, comprehensive presurgical evaluation for cases of suspected CNS lymphoma.
中枢神经系统（CNS）淋巴瘤的诊断颇具挑战。目前，对于哪些病例需要进行术前评估以及对于无法手术的病变应如何处理，尚无共识。我们对 CNS 淋巴瘤的诊断文献（1966 年至 2011 年 10 月）进行了回顾，以确定是否可以制定一个通用的诊断方案。我们提取了有关脑和全身影像学、血清和脑脊液（CSF）检查、眼科检查和组织活检在 CNS 淋巴瘤诊断中的作用的数据。在诊断时，影像学上的增强非常敏感：在免疫功能正常的淋巴瘤中为 98.9%，在人类免疫缺陷病毒相关性 CNS 淋巴瘤中为 96.1%。CSF 细胞学的敏感性较低（2%-32%），但与流式细胞术结合后敏感性会提高。CSF 乳酸脱氢酶同工酶 5、β2-微球蛋白和免疫球蛋白重链重排研究提高了 CSF 细胞学的敏感性（58%-85%），但特异性仅为中等（85%）。蛋白质组学和 microRNA 分析等新技术在 CNS 淋巴瘤的诊断中有超过 95%的特异性。阳性 CSF 细胞学、玻璃体活检或脑/软脑膜活检仍然是目前诊断 CNS 淋巴瘤的标准。这里概述的一种分步系统方法可能有助于快速、全面地对疑似 CNS 淋巴瘤的病例进行术前评估。
