Neoplastic angioendotheliosis: a clinicopathological entity with multifocal presentation

Abstract

Neoplastic angioendotheliosis is a rare disorder characterized by intravascular neoplastic proliferation of endothelial cells within vessels of all caliber in the meninges and neuropil. Ischemic infarcts of [human] brain and spinal cord result from occlusion of the lumina by neoplastic cells or fibrin thrombi. Transition from reactive to neoplastic endothelium can be identified in many vessels. Steroid therapy can be beneficial in reduction of severity of symptoms, but cannot alter the course of disease. Therapeutic intervention must be undertaken promptly after the diagnosis is confirmed by meningeal and cortical biopsy if the inexorable course of the disease is to be altered.