NDLI: Histologically Benign, Clinically Aggressive: Progressive Non-Optic Pathway Pilocytic Astrocytomas in Adults with NF1

Content Provider	World Health Organization (WHO)-Global Index Medicus
Author	Strowd, Roy E. Rodriguez, Fausto J. McLendon, Roger E. Vredenburgh, James J. Chance, Aaron B. Jallo, George Olivi, Alessandro Ahn, Edward S. Blakeley, Jaishri O.
Description	Author Affiliation: Strowd RE ( Department of Neurology, Johns Hopkins Hospital, Baltimore, Maryland.); Rodriguez FJ ( Department of Pathology, Johns Hopkins Hospital, Baltimore, Maryland.); McLendon RE ( Division of Neurology, Department of Pathology, Duke University Medical Center, Durham, North Carolina.); Vredenburgh JJ ( Division of Neurology, Department of Internal Medicine, Duke University Medical Center, Durham, North Carolina.); Chance AB ( Department of Neurology, Johns Hopkins Hospital, Baltimore, Maryland.); Jallo G ( Department of Neurosurgery, Johns Hopkins Hospital, Baltimore, Maryland.); Olivi A ( Department of Neurosurgery, Johns Hopkins Hospital, Baltimore, Maryland.); Ahn ES ( Department of Neurosurgery, Johns Hopkins Hospital, Baltimore, Maryland.); Blakeley JO ( Department of Neurology, Johns Hopkins Hospital, Baltimore, Maryland.)
Abstract	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 <18) with clinically or radiographically progressive extra-optic PAs. Demographic, clinical, histologic, and radiographic data were collected. Three adult NF1 cases and four pediatric NF1 controls were identified. Mean age was 32.3 ± 9.5 years, 66% male (cases); 12.8 ± 4.2 years, 100% male (controls). Symptomatic progression occurred in two-of-three adults (67%) while the majority of pediatric patients presented with isolated radiographic progression (n = 3, 75%). Onset tended to be more rapid in adults (4 ± 1 vs. 14 ± 8.3 months, P = 0.10). Subtotal resection was the treatment for all pediatric patients. Radiotherapy (n = 2), chemotherapy (n = 2), and targeted, biologic agents (n = 2) were administered in adults. Although all pediatric patients are living, outcomes were universally poor in adults with progression to death in all (median survival 17.1 months, range 6.6-30.3). In conclusion, despite grade I histology, all three adult NF1 patients with progressive extra-optic PAs suffered an aggressive clinical course which was not seen in pediatric patients. Clinicians should be aware of this clinico-histologic discrepancy when counseling and managing adult NF1 patients with progressive extra-optic PAs. © 2016 Wiley Periodicals, Inc.
File Format	HTM / HTML
ISSN	15524825
e-ISSN	15524833
DOI	10.1002/ajmg.a.37622
Journal	American Journal of Medical Genetics Part A
Issue Number	6
Volume Number	170
Language	English
Publisher	Wiley-Blackwell
Publisher Date	2016-06-01
Publisher Place	United States
Access Restriction	Open
Subject Keyword	Human genetics Medical Genetics Genetics
Content Type	Text
Resource Type	Article
Subject	Genetics Genetics (clinical)

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