Central Nervous System Involvement in Congenital Visceral Fibromatosis

Abstract

Congenital visceral myofibromatosis is an uncommon disorder characterized by multiple tumors of myofibroblastic origin in the neonatal period. The natural history of the disorder has been well delineated. The myofibroblast is the cell of origin of the tumor. This is a report of a patient in whom multiple mesenchymal tumors occurred in the CNS as well as in other organs. Light and electron microscopic findings of the CNS lesions are similar to those of the somites and viscera.