Angioendotheliomatosis of the nose with fatal systemic dissemination

Angioendotheliomatosis is a rare intravascular neoplastic process which has been presumed to be of endothelial origin. A 62-year-old woman had manifestations of this disorder which were initially localized to the nasal region, followed by fatal systemic dissemination. Electron microscopic examination of the neoplastic cells disclosed structures interpreted as Weibel-Palade bodies, lending some support to a theory of endothelial histogenesis. Forty cases of angioendotheliomatosis have been previously reported, providing data that help define this disease process as a distinct clinicopathologic entity.