Mutation status and epithelial differentiation stratify recurrence risk in chordoid meningioma—a multicenter study with high prognostic relevance

Maria Magdalena Georgescu, Anil Nanda, Yan Li, Bret C. Mobley, Phyllis L. Faust, Jack M. Raisanen, Adriana Olar

Research output: Contribution to journalArticle

1 Scopus citations

Abstract

Chordoid meningioma is a rare WHO grade II histologic variant. Its molecular alterations or their impact on patient risk stratification have not been fully explored. We performed a multicenter, clinical, histological, and genomic analysis of chordoid meningiomas from 30 patients (34 tumors), representing the largest integrated study to date. By NHERF1 microlumen immunohistochemical detection, three epithelial differentiation (ED) groups emerged: #1/fibroblastic-like, #2/epithelial-poorly-differentiated and #3/epithelial-well-differentiated. These ED groups correlated with tumor location and genetic profiling, with NF2 and chromatin remodeling gene mutations clustering in ED group #2, and TRAF7 mutations segregating in ED group #3. Mutations in LRP1B were found in the largest number of cases (36%) across ED groups #2 and #3. Pathogenic ATM and VHL germline mutations occurred in ED group #3 patients, conferring an aggressive or benign course, respectively. The recurrence rate significantly correlated with mutations in NF2, as single gene, and with mutations in chromatin remodeling and DNA damage response genes, as groups. The recurrence rate was very high in ED group #2, moderate in ED group #3, and absent in ED group #1. This study proposes guidelines for tumor recurrence risk stratification and practical considerations for patient management.

Original languageEnglish (US)
Article number225
JournalCancers
Volume12
Issue number1
DOIs
StatePublished - Jan 2020

Keywords

  • ATM
  • Chordoid meningioma
  • Chromatin remodeling genes
  • Epithelial differentiation
  • NF2
  • NHERF1/EBP50
  • TRAF7

ASJC Scopus subject areas

  • Oncology
  • Cancer Research

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