Abstract
High-grade gliomas in patients with neurofibromatosis type 1 are rare and may therefore not be considered in the differential of brain lesions. Here, we describe 5 children with neurofibromatosis type 1; four of them developed various types of high-grade gliomas. The fifth patient had imaging features concerning for a high-grade lesion, but tissue diagnosis showed a low-grade glioma. The cases and literature summary provided here are to raise awareness for the occurrence of high-grade gliomas in children with neurofibromatosis type 1 and the limited ability of imaging features alone to predict a high-grade malignancy.