Neuroepithelial Tumor Pathology and Imaging

Neuroepithelial tumours constitute a diverse group of central nervous system neoplasms arising from glial or neuronal precursors. Contemporary classification integrates histological features with molecular diagnostics, including DNA methylation profiling, gene‐fusion detection and targeted sequencing, following the World Health Organization’s 2021 scheme. Pathological hallmarks range from low‐grade glioneuronal lesions with mixed neuronal and oligodendroglial differentiation to high‐grade diffuse gliomas driven by receptor tyrosine kinase or MAPK pathway alterations. Imaging plays a pivotal role in diagnosis and management: conventional magnetic resonance imaging (MRI) delineates lesion extent and structural characteristics, while advanced techniques—diffusion‐weighted imaging, perfusion MRI and metabolic PET—provide insights into cellular density, vascularity and metabolic activity. Correlation of histopathology and imaging phenotypes refines grading, guides surgical planning and may predict therapeutic response. Recent advances have emphasised the stabilising role of epigenetic signatures in stratifying indolent “leave‐me‐alone” lesions from more aggressive gliomas, underscoring the global significance of integrated diagnostics and the potential for tailored therapeutic interventions.

Research from Nature Portfolio

Recent studies have elucidated a novel mechanism of oncogenic activation in myxoid glioneuronal tumours harbouring PDGFRA K385I/L mutations. Functional assays demonstrate that substitution of lysine at codon 385 stabilises PDGFRα in an active dimeric conformation, leading to ligand-independent activation of downstream STAT and AKT signalling. Biophysical interaction studies corroborate enhanced receptor dimerisation, while pharmacological evaluation reveals pronounced sensitivity of mutant receptors to imatinib, dasatinib and avapritinib. These findings not only clarify the molecular basis of PDGFRA‐driven tumourigenesis in this glioneuronal subtype but also identify actionable targets for precision therapy, informing future trials of tyrosine kinase inhibitors in low-grade neuroepithelial tumours.

Neuroepithelial Tumor Pathology and Imaging publication trend

The graph below shows the total number of articles in neuroepithelial tumor pathology and imaging across all publications each year (not limited to Nature Index journals).

Technical terms

Neuroepithelial tumour: Neoplasm derived from neural or glial precursor cells in the central nervous system.

Glioneuronal tumour: Tumour containing both glial and neuronal cellular components with mixed differentiation.

Oligodendrocyte precursor cell (OPC): Early progenitor that differentiates into myelin-producing oligodendrocytes.

DNA methylation profiling: Genome-wide assay of epigenetic marks used for tumour classification and prognostication.

MRI FLAIR: Fluid-attenuated inversion recovery sequence that suppresses cerebrospinal fluid to highlight parenchymal lesions.

Tyrosine kinase inhibitor (TKI): Small molecule drug that blocks receptor tyrosine kinases involved in oncogenic signalling.

References

1. Pediatric CNS tumors and 2021 WHO classification: what do oncologists need from pathologists?. Frontiers in Molecular Neuroscience (2024).
2. PDGFRA K385 mutants in myxoid glioneuronal tumors promote receptor dimerization and oncogenic signaling. Scientific Reports (2024).
3. Diffuse glioneuronal tumor with oligodendroglioma-like features and nuclear clusters (DGONC), new name and new problems: an illustration of one case with atypical morphology and biology. Acta Neuropathologica Communications (2024).
4. Enrichment of oligodendrocyte precursor phenotypes in subsets of low-grade glioneuronal tumours. Brain Communications (2024).
5. Multinodular and Vacuolating Neuronal Tumors: Imaging Features, Diagnosis, and Management Challenges. Diagnostics (2025).