LETHAL MANIFESTATIONS OF NEUROFIBROMATOSIS TYPE-1 IN CHILDHOOD

Neurofibromatosis type 1 (NF-1), also known as peripheral neurofibromatosis or von Recklinghausen's disease, is a common inherited neurocutaneous syndrome. Complications of the disease are numerous and may be fatal, but because the frequency of potentially lethal complications increases with age, deaths from the disease are often thought to occur only in adults. Examination of autopsy records at the Adelaide Children's Hospital for the period 1952 to 1991 revealed four cases of children with NF-1. These cases illustrate a number of interesting pathologic features including a proliferative glial infiltrate involving the subarachnoid space, two primary gliomas within the same patient, gliomatosis cerebri, and sudden death due to massive intratumoral hemorrhage.