Department of Neurology, Showa University School of Medicine, Hatanodai, Shinagawa-ku, Tokyo, Japan.
Neuropathology. 2009 Dec;29(6):704-7. doi: 10.1111/j.1440-1789.2009.01004.x. Epub 2009 Mar 3.
A 59-year-old immunocompetent man was admitted to our hospital because of progressive dementia with concomitant bilateral uveitis. The first brain MRI revealed diffuse hyperintense lesions in the cerebral white matter of both hemispheres on a T2-weighted image and fluid-attenuated inversion recovery image. However, another MRI taken more than 1 month later revealed enhanced cohesive mass lesions in the bilateral thalami, in addition to the white matter lesions. The white matter lesions were slightly hyperintense on a diffusion-weighted image and apparent diffusion coefficient map image, suggesting vasogenic edema. One year after the onset of uveitis, he died of respiratory failure. Pathological diagnosis was diffuse large B-cell lymphoma with perivascular proliferation and diffuse scattered infiltration in the cerebrum and brainstem. Microscopically, cohesive mass lesions in the bilateral thalami were a massive cluster of lymphoma cells. This is a case of primary CNS lymphoma (PCNSL) mimicking 'lymphomatosis cerebri (LC)' at first but later exhibiting typical mass lesions, giving rise to the possibility that cases of LC might unmask features of regular lymphomas in their later course more often than believed thus far.
一位 59 岁免疫功能正常的男性因进行性痴呆伴双侧葡萄膜炎而入院。首次脑部 MRI 显示 T2 加权图像和液体衰减反转恢复图像上双侧大脑半球脑白质弥漫性高信号病变。然而,1 个多月后进行的另一次 MRI 显示除了白质病变外,双侧丘脑还有增强的凝聚性肿块病变。弥散加权图像和表观弥散系数图上的白质病变呈轻度高信号,提示血管源性水肿。葡萄膜炎发病 1 年后,他因呼吸衰竭而死亡。病理诊断为弥漫性大 B 细胞淋巴瘤,伴有血管周围增殖和大脑及脑干弥漫性散在浸润。显微镜下,双侧丘脑的凝聚性肿块病变是大量淋巴瘤细胞的聚集。这是一例原发性中枢神经系统淋巴瘤(PCNSL),最初表现为“淋巴瘤病(LC)”,但后来表现出典型的肿块病变,这使得 LC 病例在其病程后期更可能表现出常规淋巴瘤的特征,比目前认为的更为常见。