The Vascular Lesions of Neurofibromatosis

Abstract

Peculiar arterial lesions were found in seven of 18 cases of neurofi bromatosis. As previously described, these were of four types: pure intimal, advanced intimal, intimal aneurysmal, and nodular. Many lesions had features of more than one of the pure types. Other characteristics of the lesions suggested that they are of Schwann cell origin. It is proposed that the pathogenesis of all of the types of arterial lesions is the same—the proliferation of Schwann cells within arteries with secondary degenerative changes, e.g., fibrosis, resulting in lesions with very different appearances.