Intracranial gliomas in neurofibromatosis type 1

Robert Listernick, Joel Charrow, David H. Gutmann*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

132 Scopus citations

Abstract

Optic pathway gliomas and brainstem gliomas are the predominant intracranial neoplasms associated with neurofibromatosis type 1 (NF1). Before the past 15 years, studies of optic pathway gliomas in NF1 were hampered by the inaccurate diagnosis of NF1, the unavailability of noninvasive neuroimaging techniques, and the frequent rendering of what would now be considered unnecessary, overly aggressive therapy. When studied systematically, these tumors behave in a much more benign fashion than their counterparts in children who do not have NF1. While they may cause symptoms in as many of 50% of cases, progression to the point where specific intervention is deemed necessary is unusual. Consequently, screening neuroimaging of asymptomatic patients is unwarranted. Because optic pathway tumors universally arise in children younger than 7 years of age, all such children should undergo yearly ophthalmologic evaluations and annual assessments of growth to monitor for signs of precocious puberty.

Original languageEnglish (US)
Pages (from-to)38-44
Number of pages7
JournalAmerican Journal of Medical Genetics - Seminars in Medical Genetics
Volume89
Issue number1
DOIs
StatePublished - Mar 26 1999

Keywords

  • Brainstem glioma
  • NF1
  • Optic pathway tumor
  • Precocious puberty

ASJC Scopus subject areas

  • Genetics(clinical)
  • Neuropsychology and Physiological Psychology
  • Neuroscience(all)

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