The role of surgical biopsy in the diagnosis of glioma in individuals with neurofibromatosis-1.
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.
Leonard
J R
JR
Department of Neurology, St. Louis Children's Hospital and Washington University School of Medicine, 660 S. Euclid Avenue, St. Louis, MO 63110, USA.
Perry
A
A
Rubin
J B
JB
King
A A
AA
Chicoine
M R
MR
Gutmann
D H
DH
D000293:Adolescent; D000328:Adult; D001254:Astrocytoma / Q000175:diagnosis / Q000209:etiology* / Q000473:pathology*; D001706:Biopsy / Q000592:standards; D001921:Brain / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005910:Glioma / Q000175:diagnosis / Q000209:etiology* / Q000473:pathology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009456:Neurofibromatosis 1 / Q000150:complications*