Nakamigawa T, Ishihara N, Yunoki K, Joshita T
Department of Neurology, Mihara Memorial Hospital.
Rinsho Shinkeigaku. 1990 Nov;30(11):1256-60.
The case of a 59-year-old man who was diagnosed as having neoplastic angioendotheliosis by biopsy of a small hemangioma on the skin is reported. The clinical features were characterized by hypersomnia, memory disturbance, disorientation to time and mild left hemiplegia including the face. Laboratory findings showed an elevated erythrocyte sedimentation rate, increased serum LDH, increased CSF protein and pleocytosis in the CSF. The CSF level of IgG was also elevated and was associated with the appearance of oligoclonal IgG bands. The biopsy specimen of the hemangioma on the skin revealed that some small vessels were packed with atypical mononuclear cells which were positive for anti-B cell antibody. Magnetic resonance imaging (MRI) of the brain detected multiple lesions located in the cerebellum, thalamus and caudate nucleus. The left paramedian thalamic lesion might be responsible for his characteristic mood and behavioral changes. The serial MRI study disclosed that some lesions progressively enlarged and duplicated in number. These findings might be typical for neoplastic angioendotheliosis, in which the rapidly proliferating cells occluded small vessels one after another in the central nervous system. The serial study of MRI may serve an important diagnostic purpose in this disease, although most patients with this disease, so far, have been diagnosed by autopsy.
报告了一例59岁男性病例，该患者通过皮肤小血管瘤活检被诊断为肿瘤性血管内皮增生症。临床特征表现为嗜睡、记忆障碍、时间定向障碍以及包括面部在内的轻度左侧偏瘫。实验室检查结果显示红细胞沉降率升高、血清乳酸脱氢酶升高、脑脊液蛋白增加以及脑脊液中细胞增多。脑脊液中IgG水平也升高，并伴有寡克隆IgG带出现。皮肤血管瘤的活检标本显示，一些小血管内充满了抗B细胞抗体阳性的非典型单核细胞。脑部磁共振成像（MRI）检测到位于小脑、丘脑和尾状核的多个病灶。左侧丘脑正中旁病灶可能是其特征性情绪和行为改变的原因。系列MRI研究显示，一些病灶逐渐增大且数量增多。这些发现可能是肿瘤性血管内皮增生症的典型表现，即快速增殖的细胞在中枢神经系统中一个接一个地阻塞小血管。MRI的系列研究在这种疾病中可能具有重要的诊断意义，尽管迄今为止大多数该疾病患者是通过尸检确诊的。
