Document Detail


Aggressive phenotypic and genotypic features in pediatric and NF2-associated meningiomas: a clinicopathologic study of 53 cases.
MedLine Citation:
PMID:  11589430     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Pediatric and NF2-associated meningiomas are uncommon and poorly characterized in comparison to sporadic adult cases. In order to elucidate their molecular features, we analyzed MIB-1, progesterone receptor (PR), NF2, merlin, DAL-1, DAL-1 protein, and chromosomal arms 1p and 14q in 53 meningiomas from 40 pediatric/NF2 patients using immunohistochemistry and dual-color fluorescence in situ hybridization (FISH). Fourteen pediatric (42%) patients, including 5 previously undiagnosed patients, had NF2. The remaining 19 (58%) did not qualify. All 7 of the adult patients had NF2. Meningioma grading revealed 21 benign (40%), 26 atypical (49%), and 6 anaplastic (11%) examples. Other aggressive findings included high mitotic index (32%), high MIB-1 LI (37%), aggressive variant histology (e.g. papillary, clear cell) (25%), brain invasion (17%), recurrence (39%), and patient death (17%). FISH analysis demonstrated deletions of NF2 in 82%, DAL-1 in 82%, 1p in 60%, and 14q in 66%. NF2-associated meningiomas did not differ from sporadic pediatric tumors except for a higher frequency of merlin loss in the former (p = 0.020) and a higher frequency of brain invasion in the latter (p = 0.007). Thus, although pediatric and NF2-associated meningiomas share the common molecular alterations of their adult, sporadic counterparts, a higher fraction are genotypically and phenotypically aggressive. Given the high frequency of undiagnosed NF2 in the pediatric cases, a careful search for other features of this disease is warranted in any child presenting with a meningioma.
Authors:
A Perry; C Giannini; R Raghavan; B W Scheithauer; R Banerjee; L Margraf; D C Bowers; R A Lytle; I F Newsham; D H Gutmann
Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  Journal of neuropathology and experimental neurology     Volume:  60     ISSN:  0022-3069     ISO Abbreviation:  J. Neuropathol. Exp. Neurol.     Publication Date:  2001 Oct 
Date Detail:
Created Date:  2001-10-08     Completed Date:  2001-10-25     Revised Date:  2007-11-14    
Medline Journal Info:
Nlm Unique ID:  2985192R     Medline TA:  J Neuropathol Exp Neurol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  994-1003     Citation Subset:  IM    
Affiliation:
Divisions of Neuropathology, Washington University School of Medicine, St. Louis, Missouri 63110-1093, USA.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adult
Astrocytoma / diagnosis,  genetics,  pathology
Child
Child, Preschool
Cohort Studies
Female
Follow-Up Studies
Genotype
Humans
Male
Membrane Proteins / biosynthesis,  contraindications
Middle Aged
Neurofibromatosis 2 / diagnosis,  genetics*,  pathology*
Phenotype
Tumor Suppressor Proteins*
Grant Support
ID/Acronym/Agency:
NS041520/NS/NINDS NIH HHS; NS35858/NS/NINDS NIH HHS
Chemical
Reg. No./Substance:
0/EPB41L3 protein, human; 0/Membrane Proteins; 0/Tumor Suppressor Proteins

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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