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Gliomas presenting after age 10 in individuals with neurofibromatosis type 1 (NF1)

From the Department of Neurology (Dr. Gutmann), Washington University School of Medicine, St. Louis, MO; National Center on Birth Defects and Developmental Disabilities (Dr. Rasmussen), Centers for Disease Control and Prevention, Atlanta, GA; Department of Dermatology (Dr. Wolkenstein), Henri-Mondor Hospital (AP-HP), Paris XII University, Créteil, France; Neuroscience Center MGH East (Dr. MacCollin), Charlestown, MA; Department of Neurosurgery (Dr. Guha), University of Toronto, Ontario, Canada; Radiation Epidemiology Branch (Dr. Inskip), Division of Cancer Epidemiology and Genetics, National Cancer Institute, Bethesda, MD; Department of Paediatrics and Child Health (Dr. North), University of Sydney, Australia; Department of Medical Genetics (Dr. Poyhonen), Family Federation of Finland, Helsinki; and Department of Medical Genetics (P.H. Birch and Dr. Friedman), University of British Columbia, Vancouver, Canada.

Address correspondence and reprint requests to Dr. David H. Gutmann, Department of Neurology, Washington University School of Medicine, Box 8111, 660 S. Euclid Avenue, St. Louis, MO 63110; e-mail: gutmannd@ neuro.wustl.edu

Children with neurofibromatosis 1 (NF1) often develop low-grade gliomas, but brain tumors are infrequently encountered in adults with NF1. The authors present evidence from two clinical series, one including patients known to have NF1 and another focusing on adults with new onset brain tumors, that suggests an association between NF1 and symptomatic gliomas in older individuals. They also summarize the clinical data on 17 adolescents or adults with NF1 and symptomatic gliomas. The findings suggest that individuals with NF1 are at increased risk of developing gliomas throughout their lives.

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