Maruyama N, Ishida Y, Sato H, Koike T, Nagao K
Department of Pathology, Tokyo Metropolitan Institute of Gerontology, Japan.
Appl Pathol. 1987;5(4):246-52.
Neoplastic angioendotheliomatosis (NAE) is a rare and fatal disorder and has been regarded as a multifocal in situ neoplastic change of endothelial cells. This report describes a case of NAE from whom a biopsy specimen was obtained and studied immunohistologically using several monoclonal antibodies against lymphocyte-associated antigens. The tumor cells occluding luminal space express both T and B cell markers such as Leu-1, Leu-3, Leu-10, Leu-M3, HLA-DR, and HLA-DQ, whereas they do not express Leu-2, Leu-4, lymphocyte-common antigen, and immunoglobulins. Factor VIII as a marker of vascular endothelial cell is false positive in the present case. Since immunoglobulins as a definitive B lymphocyte marker are negative in this case, there is no direct evidence indicating that NAE is a tumor of B lymphocytic lineage as previously reported. These results suggest the two possibilities that NAE is a unique variant of T cell lymphoma or true vascular endothelial tumor sharing the common histogenesis with malignant lymphoma. As it stands the former possibility is more likely than the latter in our case.
肿瘤性血管内皮瘤病（NAE）是一种罕见的致命性疾病，被认为是内皮细胞的多灶性原位肿瘤性改变。本报告描述了一例NAE病例，获取了其活检标本，并使用几种针对淋巴细胞相关抗原的单克隆抗体进行免疫组织学研究。阻塞管腔空间的肿瘤细胞表达T和B细胞标志物，如Leu-1、Leu-3、Leu-10、Leu-M3、HLA-DR和HLA-DQ，而不表达Leu-2、Leu-4、淋巴细胞共同抗原和免疫球蛋白。在本病例中，作为血管内皮细胞标志物的因子VIII呈假阳性。由于作为明确B淋巴细胞标志物的免疫球蛋白在本病例中为阴性，因此没有直接证据表明NAE如先前报道的那样是B淋巴细胞谱系的肿瘤。这些结果提示了两种可能性，即NAE是T细胞淋巴瘤的独特变体或与恶性淋巴瘤具有共同组织发生的真正血管内皮肿瘤。就目前情况而言，在我们的病例中，前一种可能性比后一种更有可能。
