Neurofibromatosis type 1 (NF1) is an inherited autosomal dominant disorder characterized by numerous cutaneous features, including cafe-au-lait macules, skinfold freckling and iris hamartomas. In addition, individuals with NF1 are prone to the development of both benign and malignant tumors. The most common CNS tumor in children and adults with NF1 is the glioma. In childhood, gliomas are primarily located in the optic pathway, and less frequently in the hypothalamus and brainstem. Regular ophthalmologic evaluations in children are essential for the effective management of these tumors in patients with NF1. Adults, in contrast, are more likely to develop higher grade gliomas, which are treated in a similar fashion as their sporadic counterparts.

Original languageEnglish
Pages (from-to)535-539
Number of pages5
JournalExpert Review of Neurotherapeutics
Issue number4
StatePublished - Apr 2009


  • Astrocytoma
  • Brain tumor
  • Brainstem glioma
  • Optic glioma
  • Pilocytic astrocytoma


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