NEOPLASTIC ANGIOENDOTHELIOMATOSIS Report of Two Autopsy Cases with Special Reference to the Origin of Atypical Cells

Abstract

Two autopsy cases of neoplastic angioendotheliomatosls (NAE) were presented. Both patients were elderly woman, characterized by an ascending progression of transverse myelopathy and abnormal brain shadows similar to metastatic tumor in computer tomographic examination in Case 1, and by typical clinical features of cerebral infarction in Case 2. Postmortem examinations of both cases revealed an exclusive intravascular distribution of large atypical cells in generalized organs, especially in the central nervous system, and associated multiple cerebral infarcts and, in Case 1, widespread demyelina‐tion of the spinal cord. There were no distinct lesions suggestive of a primary focus. In both cases only a few atypical cells were immunohistochemically positive for factor VHI‐related antigen, likely due to non‐specific absorption of serum factor VIII into the cells. On the other hand, almost all of the atypical cells were immunoreactive for LN‐1, LN‐2, and leukocyte common antigen, suggestive of lymphocytic (B cell) origin.

Intravascular lymphomatosis (neoplastic angioendotheliosis) of the central nervous system: case report and literature review

“…Symptoms consistent with frontal lobe involvement are often recorded [2,28]. Focal neurologic symptoms are also very frequent [7,32,34,40,44]. They are often multifocal and recurrent, and suggest stroke [47] or transient ischemic attack [28] rather than brain tumor [39].…”

“…Although the clinical manifestations of ILCNS are diverse, the commonest clinical picture is a subacute 'vascular dementia' [2,43,44]. More rarely, patients present with an isolated dementia [25], with a progressive focal deficit suggesting brain tumor [26] or with isolated stroke [40] or myelopathy [41]. In addition, about half of the patients also have constitutional symptoms such as fever, severe weight loss and asthenia.…”

“…Elevation in LDH is not a common feature of the other conditions that may minic ILCNS, increasing the usefulness of this test in making the diagnosis. It is noteworthy that circulating neoplastic cells were identified in only 2 cases [40,45]. Electroencephalography was abnormal in every patient in whom it was recorded, showing non specific abnormalities such as diffuse, focal or multifocal slow waves.…”

“…In i case, the diagnosis was established by renal and liver biopsies [25]. In three other patients, malignant cells were found in biopsy of tumorous adrenals revealed by CT-scan [28], in lymph node biopsy [45] or in blood specimen [40], but the accurate diagnosis was not established. Macroscopic findings consist in numerous ischemic foci often associated with large areas of infarction.…”

“…Some cases have been reported in which the patient had a previous history of lymphoma [12], or was subsequently found to have a lymphoma constituted by the same cells as ILCNS [13]. Nearly all the recent reported cases have demonstrated a strongly positive staining for Leucocyte Common Antigen (LCA) [2,4,5,16,31,32,40,47,[49][50][51], which is immunospecific for reactive and neoplastic leukocyte proliferation, and is unadsorbable [2]. The few studies including ours using a large panel of antisera have shown that the lymphomatous proliferation was, in most cases, of B-lineage [2-4, 16, 26, 40].…”