Chen Lingxu, Wang Xiaochen, Wang Sihui, Sun Shengjun
Department of Radiology, Beijing Tiantan Hospital, Capital Medical University, No.119 South Fourth Ring West Road, Fengtai District, Beijing, 100070, P.R. China.
Department of Radiology, Beijing Neurosurgical Institute, No.119 South Fourth Ring West Road, Fengtai District, Beijing, 100070, P.R. China.
Neurol Sci. 2025 Apr 25. doi: 10.1007/s10072-025-08099-6.
Intravascular large B-cell lymphoma (IVLBCL) of the central nervous system (CNS) is a rare subtype of diffuse large B-cell lymphoma. Diagnosing CNS-limited IVLBCL is particularly challenging due to its non-specific clinical presentation and laboratory findings. We present three cases of CNS-limited IVLBCL, expanding the understanding of its imaging spectrum and enhancing preoperative diagnostic accuracy.
Case 1 involved a 58-year-old male presenting with mild neurological symptoms, initially misdiagnosed as cerebrovascular disease. MRI showed patchy areas of diffusion restriction adjacent to the posterior horn of the left lateral ventricle, progressing over four months to a patchy hemorrhagic lesion in the right temporoparietal region with leptomeningeal enhancement. Case 2 described a 53-year-old male with progressive limb weakness and cognitive decline, initially managed as a demyelinating disease. MRI demonstrated extensive white matter hyperintensities and multiple areas of diffusion restriction. Case 3 involved a 67-year-old female with recurrent cognitive decline and gait disturbance. MRI findings included patchy T2 hyperintensities in the white matter, gyrus-like hyperintensity on T1WI, and punctate and linear enhancement. Cases 1 and 2 were diagnosed as IVLBCL post-biopsy, while in Case 3, IVLBCL was considered based on imaging features and differentiated from CNS vasculitis.
These cases highlight the variable and often non-specific MRI characteristics of CNS-IVLBCL, including infarct-like lesions, persistent diffusion restriction, and SWI abnormalities. Early recognition of these imaging findings can aid in timely diagnosis and treatment, potentially improving patient outcomes.
中枢神经系统（CNS）血管内大B细胞淋巴瘤（IVLBCL）是弥漫性大B细胞淋巴瘤的一种罕见亚型。由于其非特异性的临床表现和实验室检查结果，诊断中枢神经系统局限型IVLBCL极具挑战性。我们报告3例中枢神经系统局限型IVLBCL病例，以拓展对其影像学表现谱的认识并提高术前诊断准确性。
病例1为一名58岁男性，表现为轻度神经症状，最初被误诊为脑血管疾病。MRI显示左侧脑室后角旁有斑片状扩散受限区域，4个月内进展为右侧颞顶叶区域的斑片状出血性病变并伴有软脑膜强化。病例2为一名53岁男性，有进行性肢体无力和认知功能减退，最初按脱髓鞘疾病治疗。MRI显示广泛的白质高信号和多个扩散受限区域。病例3为一名67岁女性，有反复的认知功能减退和步态障碍。MRI表现包括白质内斑片状T2高信号、T1WI上的脑回样高信号以及点状和线状强化。病例1和病例2活检后诊断为IVLBCL，而病例3根据影像学特征考虑为IVLBCL，并与中枢神经系统血管炎相鉴别。
这些病例突出了中枢神经系统IVLBCL多变且常为非特异性的MRI特征，包括梗死样病变、持续的扩散受限和磁敏感加权成像（SWI）异常。早期识别这些影像学表现有助于及时诊断和治疗，可能改善患者预后。
