Kanai Ryuichi, Shibuya Makoto, Hata Takashi, Hori Makoto, Hirabayashi Kenichi, Terada Tadashi, Fujii Koji
Department of Neurosurgery, Shizuoka City Shimizu Hospital, Shizuoka, Japan.
J Neurooncol. 2008 Jan;86(1):83-8. doi: 10.1007/s11060-007-9437-9. Epub 2007 Jul 5.
'Lymphomatosis cerebri' (LC) is a term indicating a diffusely infiltrating form of primary central nervous system lymphoma (PCNSL) without evidence of a mass lesion. Not infrequently, diagnostic confusion is caused by its presentation on cranial magnetic resonance images (MRI), which is characterized by diffuse leukoencephalopathy without contrast enhancement. In this report, we describe a 53-year-old, immunocompetent man who had an insidiously progressive dementia and right weakness. On serial MRI in 4 months duration, diffuse white matter lesions without contrast enhancement gradually progressed, which was clinically consistent with his worsening condition. Biopsy specimen demonstrated nondestructive, diffusely infiltrating, large B-cell lymphoma, diagnosing LC. After the biopsy, corticosteroids were initiated, which dramatically alleviated his symptoms. Afterwards, he was treated by whole brain irradiation (total 36Gy) and discharged without noticeable deficits. Diagnosis of LC requires additional examinations generally not performed in the other white matter disorders. In suspected cases, biopsy should be performed to avoid deferring adequate cytostatic treatment.
“脑淋巴瘤病”(LC)是一个术语,用于表示原发性中枢神经系统淋巴瘤(PCNSL)的一种弥漫性浸润形式,且无占位性病变证据。其在颅脑磁共振成像(MRI)上的表现常导致诊断混淆,其特征为弥漫性白质脑病且无强化。在本报告中,我们描述了一名53岁免疫功能正常的男性,他隐匿性地逐渐出现痴呆和右侧肢体无力。在4个月的系列MRI检查中,无强化的弥漫性白质病变逐渐进展,这在临床上与他病情的恶化相一致。活检标本显示为非破坏性、弥漫性浸润的大B细胞淋巴瘤,诊断为LC。活检后开始使用皮质类固醇,这显著缓解了他的症状。之后,他接受了全脑照射(总计36Gy)并出院,无明显缺陷。LC的诊断需要进行一些通常在其他白质疾病中不进行的额外检查。在疑似病例中,应进行活检以避免延迟适当的细胞毒性治疗。