SPECIAL ISSUES IN THE MANAGEMENT OF GLIOMAS IN CHILDREN WITH NEUROFIBROMATOSIS-1

Neurofibromatosis 1 (NF1) is a common multisystem disorder that is fre quently associated with neoplastic and non-neoplastic lesions within t he central nervous system. Improvements in neuroimaging have led to in creased detection of both types of lesions. Focal areas of increased T 2 signal represent the most common abnormalities detected. The vast ma jority of such lesions are non-neoplastic and fluctuate in number and size during childhood. Optic pathway tumors are second in frequency an d generally manifest an indolent natural history, although some lesion s will increase in size over time and lead to progressive visual impai rment. A smaller percentage of patients will develop gliomas within th e cerebral and cerebellar hemispheres of brainstem. This article will review areas of controversy in the evaluation and follow-up of patient s with NF1 and will present: our approachh to these issues. We will al so discuss therapeutic considerations in these patients that take into account the unique features of the underlying disorder.