PMID- 11589430
OWN - NLM
STAT- MEDLINE
DCOM- 20011025
LR  - 20190513
IS  - 0022-3069 (Print)
IS  - 0022-3069 (Linking)
VI  - 60
IP  - 10
DP  - 2001 Oct
TI  - Aggressive phenotypic and genotypic features in pediatric and NF2-associated 
      meningiomas: a clinicopathologic study of 53 cases.
PG  - 994-1003
AB  - 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.
FAU - Perry, A
AU  - Perry A
AD  - Divisions of Neuropathology, Washington University School of Medicine, St. Louis, 
      Missouri 63110-1093, USA.
FAU - Giannini, C
AU  - Giannini C
FAU - Raghavan, R
AU  - Raghavan R
FAU - Scheithauer, B W
AU  - Scheithauer BW
FAU - Banerjee, R
AU  - Banerjee R
FAU - Margraf, L
AU  - Margraf L
FAU - Bowers, D C
AU  - Bowers DC
FAU - Lytle, R A
AU  - Lytle RA
FAU - Newsham, I F
AU  - Newsham IF
FAU - Gutmann, D H
AU  - Gutmann DH
LA  - eng
GR  - NS041520/NS/NINDS NIH HHS/United States
GR  - NS35858/NS/NINDS NIH HHS/United States
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PT  - Research Support, U.S. Gov't, P.H.S.
PL  - England
TA  - J Neuropathol Exp Neurol
JT  - Journal of neuropathology and experimental neurology
JID - 2985192R
RN  - 0 (EPB41L3 protein, human)
RN  - 0 (Membrane Proteins)
RN  - 0 (Microfilament Proteins)
RN  - 0 (Tumor Suppressor Proteins)
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Astrocytoma/diagnosis/genetics/pathology
MH  - Child
MH  - Child, Preschool
MH  - Cohort Studies
MH  - Contraindications
MH  - Female
MH  - Follow-Up Studies
MH  - Genotype
MH  - Humans
MH  - Male
MH  - Membrane Proteins/biosynthesis
MH  - Microfilament Proteins
MH  - Middle Aged
MH  - Neurofibromatosis 2/diagnosis/*genetics/*pathology
MH  - Phenotype
MH  - *Tumor Suppressor Proteins
EDAT- 2001/10/09 10:00
MHDA- 2001/10/26 10:01
CRDT- 2001/10/09 10:00
PHST- 2001/10/09 10:00 [pubmed]
PHST- 2001/10/26 10:01 [medline]
PHST- 2001/10/09 10:00 [entrez]
AID - 10.1093/jnen/60.10.994 [doi]
PST - ppublish
SO  - J Neuropathol Exp Neurol. 2001 Oct;60(10):994-1003. doi: 10.1093/jnen/60.10.994.