Arterial Lesions Associated with Neurofibromatosis

The nature of the vascular lesions associated with neurofibromatosis is not clear. A histologic and ultrastructural study of a case of neurofibromatosis with renal lesions revealed no association of the abnormal vessels with neural malformation. The characteristic nodular aggregates of cells in the abnormal arterial wall were identified as smooth muscle. This and other changes described are suggestive of a primary mesodermal disorder. From the authors’ study of this and previously reported cases, descriptions of two basic types of vascular involvement in neurofibromatosis evolved.