EANO guideline on rational molecular testing of gliomas, glioneuronal, and neuronal tumors in adults for targeted therapy selection - Sorbonne Université Access content directly
Journal Articles Neuro-Oncology Year : 2023

EANO guideline on rational molecular testing of gliomas, glioneuronal, and neuronal tumors in adults for targeted therapy selection

David Capper
Guido Reifenberger
  • Function : Author
Pim J French
  • Function : Author
Leonille Schweizer
Michael Weller
Simone P Niclou
Philipp Euskirchen
Christine Haberler
Monika E Hegi
  • Function : Author
Sebastian Brandner
Emilie Le Rhun
  • Function : Author
Roberta Rudà
  • Function : Author
Ghazaleh Tabatabai
Felix Sahm
Patrick Y Wen
  • Function : Author
Pieter Wesseling
  • Function : Author
Matthias Preusser
Martin J van den Bent
  • Function : Author
  • PersonId : 892861

Abstract

Abstract The mainstay of treatment for adult patients with gliomas, glioneuronal and neuronal tumors consists of combinations of surgery, radiotherapy, and chemotherapy. For many systemic cancers, targeted treatments are a part of the standard of care, however, the predictive significance of most of these targets in central nervous system (CNS) tumors remains less well-studied. Despite that, there is increasing use of advanced molecular diagnostics that identify potential targets, and tumor-agnostic regulatory approvals on targets also present in CNS tumors have been granted. This raises the question of when and for which targets it is meaningful to test in adult patients with CNS tumors. This evidence-based guideline reviews the evidence available for targeted treatment for alterations in the RAS/MAPK pathway (BRAF, NF1), in growth factor receptors (EGFR, ALK, fibroblast growth factor receptor (FGFR), neurotrophic tyrosine receptor kinase (NTRK), platelet-derived growth factor receptor alpha, and ROS1), in cell cycle signaling (CDK4/6, MDM2/4, and TSC1/2) and altered genomic stability (mismatch repair, POLE, high tumor mutational burden (TMB), homologous recombination deficiency) in adult patients with gliomas, glioneuronal and neuronal tumors. At present, targeted treatment for BRAF p.V600E alterations is to be considered part of the standard of care for patients with recurrent gliomas, pending regulatory approval. For approved tumor agnostic treatments for NTRK fusions and high TMB, the evidence for efficacy in adult patients with CNS tumors is very limited, and treatment should preferably be given within prospective clinical registries and trials. For targeted treatment of CNS tumors with FGFR fusions or mutations, clinical trials are ongoing to confirm modest activity so far observed in basket trials. For all other reviewed targets, evidence of benefit in CNS tumors is currently lacking, and testing/treatment should be in the context of available clinical trials.
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hal-04531041 , version 1 (03-04-2024)

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David Capper, Guido Reifenberger, Pim J French, Leonille Schweizer, Michael Weller, et al.. EANO guideline on rational molecular testing of gliomas, glioneuronal, and neuronal tumors in adults for targeted therapy selection. Neuro-Oncology, 2023, 25 (5), pp.813-826. ⟨10.1093/neuonc/noad008⟩. ⟨hal-04531041⟩
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