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Perivascular NOTCH3+ Stem Cells Drive Meningioma Tumorigenesis and Resistance to Radiotherapy

  • Abrar Choudhury
  • , Martha A. Cady
  • , Calixto Hope G. Lucas
  • , Hinda Najem
  • , Joanna J. Phillips
  • , Brisa Palikuqi
  • , Naomi Zakimi
  • , Tara Joseph
  • , Janeth O. Birrueta
  • , William C. Chen
  • , Nancy A.Oberheim Bush
  • , Shawn L. Hervey-Jumper
  • , Ophir D. Klein
  • , Christine M. Toedebusch
  • , Craig M. Horbinski
  • , Stephen T. Magill
  • , Aparna Bhaduri
  • , Arie Perry
  • , Peter J. Dickinson
  • , Amy B. Heimberger
  • Alan Ashworth, Elizabeth E. Crouch, David R. Raleigh

Research output: Contribution to journalArticlepeer-review

Abstract

Meningiomas are the most common primary intracranial tumors. Treatments for patients with meningiomas are limited to surgery and radiotherapy, and systemic therapies remain ineffective or experimental. Resistance to radiotherapy is common in high-grade meningiomas and the cell types and signaling mechanisms that drive meningioma tumorigenesis and resistance to radiotherapy are incompletely understood. Here, we report that NOTCH3 drives meningi-oma tumorigenesis and resistance to radiotherapy and find that perivascular NOTCH3+ stem cells are conserved across meningiomas from humans, dogs, and mice. Integrating single-cell transcriptomics with lineage tracing and imaging approaches in genetically engineered mouse models and xenografts, we show NOTCH3 drives tumor-initiating capacity, cell proliferation, angiogenesis, and resistance to radiotherapy to increase meningioma growth and reduce survival. To translate these findings to patients, we show that an antibody stabilizing the extracellular negative regulatory region of NOTCH3 blocks meningioma tumorigenesis and sensitizes meningiomas to radiotherapy, reducing tumor growth and improving survival.

Original languageEnglish (US)
Pages (from-to)1823-1837
Number of pages15
JournalCancer discovery
Volume14
Issue number10
DOIs
StatePublished - Oct 2024

ASJC Scopus subject areas

  • Oncology

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