Hemispheric cerebral gliomas in children with NF1: arguments for a long-term follow-up

Abstract.

Background: Neurofibromatosis type 1, or peripheral neurofibromatosis, is the most form of common phakomatosis. In some instances, it can be associated with tumors of the central nervous system. Case report: We describe four cases of hemispheric cerebral glioma in children with NF1. Two of these tumors were pilocytic astrocytomas and two, ependymomas. We reviewed the literature on hemispheric cerebral gliomas in NF1 patients in an attempt to find characteristic features of and some explanations for these lesions. Conclusion: We conclude that surgical resection of these tumors should be proposed whenever possible, with due consideration for their progressive nature and the uncertainties about malignancy. We advocate yearly clinical and neuroradiological follow-up over a long period in NF1 children, since they may develop additional CNS tumors during their lifetime.