Glioneuronal and neuronal tumors: A perspective

Area of Science:

• Neuro-oncology
• Molecular Pathology

Background:

• Glioneuronal and neuronal tumors (GNTs) are low-grade neuroepithelial tumors.
• Histological diagnosis traditionally relied on neuronal differentiation.
• Genetic alterations, especially in the MAP-kinase pathway, challenge traditional classification.

Purpose of the Study:

• To highlight the need for molecular-based classification of GNTs.
• To emphasize the importance of molecular profiling for targeted therapy selection.
• To inform clinical management strategies for GNTs.

Main Methods:

• Review of histological and genetic findings in GNTs.
• Analysis of the World Health Organization (WHO) 2021 classification updates.
• Consideration of DNA methylation profiling for tumor segregation.

Main Results:

• Histological diagnosis may not correlate with genetic alterations in GNTs.
• Molecular classification is essential given the availability of MAP-kinase pathway inhibitors.
• The WHO 2021 classification incorporates molecular data for neuroepithelial tumors.

Conclusions:

• Molecular classification is crucial for accurate GNT diagnosis and treatment.
• Targeted therapies offer new treatment avenues for GNT patients.
• Avoiding aggressive treatments like chemoradiotherapy preserves tissue for future therapies.