Articles

Intracranial gliomas in neurofibromatosis type 1

  1. Robert Listernick†,
  2. Joel Charrow‡,
  3. David H. Gutmann§,*

Article first published online: 30 AUG 1999

DOI: 10.1002/(SICI)1096-8628(19990326)89:1<38::AID-AJMG8>3.0.CO;2-M

Issue

American Journal of Medical Genetics

American Journal of Medical Genetics

Special Issue: Neurofibromatosis 1

Volume 89, Issue 1, pages 38–44, 26 March 1999

Additional Information

How to Cite

Listernick, R., Charrow, J. and Gutmann, D. H. (1999), Intracranial gliomas in neurofibromatosis type 1. American Journal of Medical Genetics, 89: 38–44. doi: 10.1002/(SICI)1096-8628(19990326)89:1<38::AID-AJMG8>3.0.CO;2-M

Author Information

  1. †

    Dr. Listernick is an associate professor of pediatrics at Northwestern University Medical School. He is co-director of the Neurofibromatosis Clinic and director of the Diagnostic and Consultation Service of the Children's Memorial Hospital.

  2. ‡

    Dr. Charrow is an associate professor of pediatrics at Northwestern University Medical School. He is co-director of the Neurofibromatosis Clinic at the Children's Memorial Hospital, where hs is also the head of the Section of Clinical Genetics.

  3. §

    Dr. Gutmann serves as the chairman of the Optic Pathway Glioma Task Force and vice-chairman of the National Neurofibromatosis Foundation Clinical Care Advisory Board. Dr. Gutmann directs a research program on the molecular biology of inherited and sporadic nervous system tumors.

*Department of Neurology, Washington University School of Medicine, Box 8111, 660 South Euclid Ave., St. Louis, MO 63110

Publication History

  1. Issue published online: 25 OCT 2002
  2. Article first published online: 30 AUG 1999

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Keywords:

  • NF1;
  • brainstem glioma;
  • optic pathway tumor;
  • precocious puberty

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. Am. J. Med. Genet. (Semin. Med. Genet.) 89:38–44, 1999. © 1999 Wiley-Liss, Inc.

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