原发于中枢神经系统的淋巴瘤样肉芽肿:病例报告并文献分析

A clinicopathologic analysis of primary central nervous system lymphomatoid granulomatosis:case report and literature review

目的探讨原发于中枢神经系统的淋巴瘤样肉芽肿的临床表现、影像学及病理学特点。方法回顾分析一例原发于中枢神经系统的淋巴瘤样肉芽肿患者的临床及影像学表现、组织学及分子生物学特点,并复习相关文献。结果男性患者,57岁。表现为记忆力减退3个月,地点定向障碍,计算力和理解判断力下降。影像学检查显示双侧额叶占位性病变,呈等T_1、长T_2信号,强化不均匀。术中可见病变区域脑组织稍膨胀,表面黄染,切面组织颜色灰白,质地较软,血供较丰富。组织学表现为以血管为中心的淋巴细胞增生性病变,破坏血管壁,部分区域增生的淋巴细胞以T细胞为主,伴大量吞噬细胞浸润及星形胶质细胞反应性增生,伴小血管增生,血管壁呈玻璃样变性,呈淋巴瘤样肉芽肿结构;部分区域增生的淋巴细胞弥漫成片,B细胞所占比例升高,细胞呈明显异型性,并具有IgK链的单克隆性扩增,表现为淋巴瘤样改变,EB病毒检测阴性。结论淋巴瘤样肉芽肿作为淋巴瘤的前期病变,应该被列入中枢神经系统弥漫性及多发性病变的鉴别诊断中,原发于中枢神经系统的淋巴瘤样肉芽肿与EB病毒的相关性尚有待进一步探讨。

Objective To investigate the clinical, neuroimaging and histopathological features of primary central nervous system lymphomatoid granulomatosis （LG）. Methods The clinical manifestation, neuroimaging, histopathological and biological features of a patient with primary central nervous system LG were presented, and the related literatures were reviewed. Results A 57-year-old male presented with memory impairment, weak in orientation, calculation, apprehension and judgment for 3 months. Magnetic resonance imaging （MRI） showed space-occupying lesions in bilateral frontal lobes, with %WI isointensity and T2WI hyperintensity, and the enhancement was irregular. The lesion was slight expansive with yellow surface and gray-white section in color and soft texture and abundant blood supply. Microscopically, the lesion was characterized by angiocentric and angiodestructive lymphoproliferation, partly showed the structure of LG characterized by T cell predominant proliferation, macrophage infiltration, astrocyte activation, small vessel proliferation and hyalinization, and partly showed the structure of lymphoma characterized by diffuse atypical B cell proliferation, with lgK monoclona] production. Epstein-Barr virus （EBV） was negative. Conclusion As a precursor disease of lymphoma, LG should be considered in the differential diagnosis of both diffuse and multifocal lesions of the central nervous system. The relavance between primary central nervous system LG and EBV infection should be further discussed.