Gliomas in patients with neurofibromatosis type 1

Abstract

Neurofibromatosis type 1 (NF1) is an inherited autosomal dominant disorder characterized by numerous cutaneous features, including café-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.

Keywords::

• astrocytoma
• brainstem glioma
• brain tumor
• optic glioma
• pilocytic astrocytoma

Financial & competing interests disclosure

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

No writing assistance was utilized in the production of this manuscript.