Fulling K H, Gersell D J
Cancer. 1983 Mar 15;51(6):1107-18. doi: 10.1002/1097-0142(19830315)51:6<1107::aid-cncr2820510623>3.0.co;2-w.
Two cases of neoplastic angioendotheliomatosis are described. Both patients presented with subacute development of dementia punctuated by focal neurologic signs. Postmortem examination in both cases disclosed a vasculocentric distribution of neoplastic cells in many organs. Two characteristic histologic patterns were seen: (1) distension of vascular spaces by free-floating, intraluminal cells and (2) asymmetric, subendothelial tumor growth. Intravascular tumor and secondary vascular changes were associated with microinfarcts in involved organs. Immunohistochemical localization of factor VIII-related antigen in the neoplastic cells of one case provides evidence for their endothelial origin. The neoplastic cells in both cases exhibited similar but nonspecific ultrastructural features. In addition, dramatic fine structural changes including the acquisition of numerous pinocytotic vesicles and bundles of fine filaments with periodic densities were found in the non-neoplastic endothelial cells.
本文描述了两例肿瘤性血管内皮瘤病。两名患者均表现为亚急性起病的痴呆，并伴有局灶性神经体征。两例患者的尸检均显示许多器官中肿瘤细胞呈血管中心性分布。观察到两种特征性组织学模式：（1）血管腔内细胞漂浮导致血管腔扩张；（2）不对称的内皮下肿瘤生长。血管内肿瘤和继发性血管改变与受累器官的微梗死有关。其中一例肿瘤细胞中VIII因子相关抗原的免疫组化定位为其内皮起源提供了证据。两例的肿瘤细胞均表现出相似但非特异性的超微结构特征。此外，在非肿瘤性内皮细胞中发现了显著的细微结构变化，包括出现大量吞饮小泡和成束的具有周期性密度的细丝。
