Deletion of the NF2 region in both meningioma and juxtaposed meningioangiomatosis: case report supporting a neoplastic relationship

Pediatr Dev Pathol. Nov-Dec 2001;4(6):568-72. doi: 10.1007/s10024001-0086-2.

Abstract

We report a case of juxtaposed atypical meningioma and meningioangiomatosis (MA) in an 8-year-old boy with no clinical stigmata or family history of neurofibromatosis. We studied the proliferative activity and genetic changes in the two lesions in an attempt to define their biologic and pathogenetic relationships. The MIB-1 index was 11% in the meningioma and <1% in the MA, indicating increased proliferative activity in the meningioma. Fluorescence in situ hybridization was done for two chromosomal regions commonly deleted in meningiomas. There was loss of the neurofibromatosis 2 locus (22q12) in both the meningioma and MA. Conversely, the region of 1p32 was not deleted. Our results indicate that both the meningioma and MA arose from the same clonal process, with the meningioma probably undergoing additional, but undefined, genetic alterations that confer upon it a more proliferative potential. This loss of 22q12 in the MA raises doubt about the presumed hamartomatous nature of MA.

Publication types

  • Case Reports
  • Comparative Study

MeSH terms

  • Antigens, Nuclear
  • Child
  • Chromosomes, Human, Pair 1
  • Chromosomes, Human, Pair 22
  • Gene Deletion*
  • Genes, Neurofibromatosis 2*
  • Humans
  • In Situ Hybridization, Fluorescence
  • Ki-67 Antigen
  • Male
  • Meningeal Neoplasms / genetics*
  • Meningeal Neoplasms / pathology
  • Meninges / pathology*
  • Meningioma / genetics*
  • Meningioma / pathology
  • Neoplasm Staging
  • Nuclear Proteins / metabolism
  • Precancerous Conditions / genetics*
  • Precancerous Conditions / pathology

Substances

  • Antigens, Nuclear
  • Ki-67 Antigen
  • Nuclear Proteins