Aggressive phenotypic and genotypic features in pediatric and NF2-associated meningiomas: A clinicopathologic study of 53 cases

Arie Perry, Caterina Giannini, Ravi Raghavan, Bernd W. Scheithauer, Ruma Banerjee, Linda Margraf, Daniel C. Bowers, Richard A. Lytle, Irene F. Newsham, David H. Gutmann

Research output: Contribution to journalArticlepeer-review

152 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)994-1003
Number of pages10
JournalJournal of Neuropathology and Experimental Neurology
Issue number10
StatePublished - 2001


  • Childhood
  • Chromosome 1
  • Chromosome 14
  • DAL-1
  • Malignant tumor
  • Meningioma
  • NF2

ASJC Scopus subject areas

  • General Medicine


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