Histologically benign, clinically aggressive: Progressive non-optic pathway pilocytic astrocytomas in adults with NF1

Although optic pathway gliomas are the most common brain tumors associated with neurofibromatosis type 1 (NF1), extra‐optic gliomas occur and may behave more aggressively with outcomes that differ by age. A retrospective case‐control study was designed to describe the clinical course of adult NF1 pa...

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Bibliographic Details
Published in:American journal of medical genetics. Part A 2016-06, Vol.170A (6), p.1455-1461
Main Authors: Strowd III, Roy E., Rodriguez, Fausto J., McLendon, Roger E., Vredenburgh, James J., Chance, Aaron B., Jallo, George, Olivi, Alessandro, Ahn, Edward S., Blakeley, Jaishri O.
Format: Article
Language:English
Subjects:
Adult
Astrocytoma - diagnosis
Astrocytoma - etiology
Astrocytoma - mortality
Astrocytoma - therapy
Biopsy
Brain - pathology
brain tumor
Case-Control Studies
Combined Modality Therapy
Diagnostic Imaging
Disease Progression
extra-optic glioma
Female
Humans
Male
Neoplasm Grading
Neurofibromatosis 1 - complications
Neurofibromatosis 1 - diagnosis
neurofibromatosis type 1
pediatric
Phenotype
pilocytic astrocytoma
Retrospective Studies
Treatment Outcome
Young Adult
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Summary:Although optic pathway gliomas are the most common brain tumors associated with neurofibromatosis type 1 (NF1), extra‐optic gliomas occur and may behave more aggressively with outcomes that differ by age. A retrospective case‐control study was designed to describe the clinical course of adult NF1 patients with progressive extra‐optic pilocytic astrocytomas (PAs) and compare to a pediatric cohort. Data for patients treated at the Johns Hopkins Comprehensive Neurofibromatosis Center from 2003 to 2013 were reviewed to identify cases (adults, age >18) and controls (pediatric, age
ISSN:1552-4825
1552-4833
DOI:10.1002/ajmg.a.37622