大脑淋巴瘤病的临床病理学分析

Clinicopathological analysis of lymphomatosis cerebri

目的 探讨大脑淋巴瘤病(lymphomatosiscerebri,LC)的临床病理学特点,以期为临床诊治提供思路。方法 选取2020年1月至2022年1月首都医科大学宣武医院确诊的LC患者4例,对其临床资料、影像学及病理学特点进行回顾性分析。结果 4例患者中男3例,女1例,年龄49~63岁,平均59岁。临床表现为进行性进展的认知功能障碍、行为改变和行走不稳等,影像学检查均表现为大脑弥漫性及多发性病变,以白质受累为主,CT呈低密度影,T1WI等或低信号,T2WI及FLAIR高信号,无明显强化。3例为立体定向穿刺活检标本,1例为开颅手术切除标本。组织学诊断均为弥漫性大B细胞淋巴瘤,结合临床及影像学表现,符合原发性中枢神经系统淋巴瘤、LC。结论 LC为累及中枢神经系统的弥漫性病变,以白质受累为主,亚急性或快速进展的认知功能障碍为其显著临床特征,涉及的鉴别诊断复杂多样,立体定向穿刺活检为获得确定诊断提供了可能性,尽早明确病理诊断是确保临床进行精准治疗的关键。

Objective To investigate the clinicopathological characteristics of lymphomatosis cerebri(LC), and to provide the ideas for clinical diagnosis and treatment. Methods Four patients with LC diagnosed in Xuanwu Hospital,Capital Medical University from January 2020 to January 2022 were selected, and the clinical data, imaging and pathological characteristics were retrospectively analyzed. Results Among the four patients, there were three males and one female, aged from 49 to 63 years, with an average of 59 years. The clinical manifestrations were progressively developed cognitive disorder, behavior change and walking unstable, etc. Imaging examination showed diffuse and multifocal lesions in the cerebrum, mainly involving white matter. CT showed low density, T1WI showed iso-or low intensity, T2WI and FLAIR showed hyperintensity without significant enhancement. Three cases had stereotactic needle biopsy specimens and one case had a craniotomy specimen. The histological diagnosis was diffuse large B-cell lymphoma.Combined with clinical and imaging findings, it was consistent with primary central nervous system lymphoma and LC.Conclusions LC diffusely involves the central nervous system, especially the cerebral white matter, with subacute or rapid progression of cognitive disorder, with complicated differential diagnosis. Stereotactic puncture is required to reach a correct diagnosis, and a pathological diagnosis in time is the key point of the precise treatment.