Brain tumors in children with von Recklinghausen neurofibromatosis

We determined the frequency of brain tumors in a series of 133 children (18 years of age or younger) with von Recklinghausen neurofibromatosis who were patients at the Children's Hospital of Zurich from 1971 to 1987. Twenty-four patients (18%) were found to have intraorbital or intracranial tumors. Twenty patients (15%) had an optic-pathway glioma (OPG), including 15 (11%) with only an OPG and 5 (4%) with both an OPG and a tumor in the posterior fossa or brainstem. Another 4 patients (3%) had a tumor outside of the optic pathways. In most cases, magnetic resonance imaging was superior to computerized tomography in demonstrating these lesions. The results of this survey are comparable to those of other published reports.