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From the Departments of Neurosurgery (J.R.L., M.R.C.), Pathology and Immunology (A.P.), Pediatrics (J.B.R., A.A.K.), Division of Hematology/Oncology, and Neurology (D.H.G.), St. Louis Children's Hospital and Washington University School of Medicine, St. Louis, MO.
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{at}wustl.edu.
Most gliomas in neurofibromatosis type 1 (NF1) are pilocytic astrocytomas (PAs) of the optic pathway occurring in young children. However, some individuals develop gliomas that lack the typical NF1-associated clinical features or radiographic appearance. We identified 17 atypical presentations from a review of 100 patients with NF1-associated gliomas. Biopsy showed that 9 were not classic PAs. These data highlight the value of biopsy in NF1-associated gliomas with unusual clinical or radiographic presentations.
Additional material related to this article can be found on the Neurology Web site. Go to www.neurology.org and scroll down the Table of Contents for the October 24 issue to find the title link for this article.
Supported by Schnuck Markets, Inc. (D.H.G.).
Disclosure: The authors report no conflicts of interest.
Received February 9, 2006. Accepted in final form June 23, 2006.
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