Petito C K, Gottlieb G J, Dougherty J H, Petito F A
Ann Neurol. 1978 May;3(5):393-9. doi: 10.1002/ana.410030505.
Neoplastic angioendotheliosis is a rare disorder characterized by progressive multifocal neurological deficit and dementia. The principal pathological findings are multiple infarcts in the brain and spinal cord and the microscopical demonstration of proliferated neoplastic cells, primarily within the lumens of arteries, arterioles, and capillaries. Vessels of the central nervous system are the most severely affected and appear to be the primary site of neoplastic proliferation. Systemic intravascular and focal extravascular proliferation can also be identified. The endothelial origin of the neoplastic cell with a gradation of changes from normal to neoplastic is demonstrated by the electron microscopical study of involved meningeal vessels. Our patient and those reported in the literature all demonstrate involvement of the central nervous system. In addition, an elevated cerebrospinal fluid protein level is present in all patients for whom that information is available, and fever and renal involvement are frequently present. Steroid therapy is generally ineffective. If the disease is suspected clinically, our studies suggest that meningeal biopsy is the diagnostic procedure of choice to help determine whether chemotherapy should be instituted.
肿瘤性血管内皮细胞增生症是一种罕见的疾病，其特征为进行性多灶性神经功能缺损和痴呆。主要病理发现是脑和脊髓的多发性梗死，以及显微镜下显示肿瘤细胞增生，主要位于动脉、小动脉和毛细血管腔内。中枢神经系统的血管受影响最严重，似乎是肿瘤增殖的主要部位。也可发现全身性血管内和局灶性血管外增殖。通过对受累脑膜血管的电子显微镜研究，证实肿瘤细胞起源于内皮细胞，且有从正常到肿瘤的一系列变化。我们的患者以及文献报道的患者均显示中枢神经系统受累。此外，所有有此信息的患者脑脊液蛋白水平均升高，且常伴有发热和肾脏受累。类固醇治疗通常无效。如果临床上怀疑患有该病，我们的研究表明，脑膜活检是有助于确定是否应进行化疗的首选诊断方法。
