Abstract
Glioneuronal tumours (GNT) are uncommon neoplasms, characterised by glial and neuronal differentiation. In the 5th edition of the World Health Organization (WHO) Classification, they are grouped under the heading "Glioneuronal and neuronal tumours", which comprises fourteen different tumours, among which the diffuse glioneuronal tumour with oligodendroglioma-like cells and nuclear clusters (DGONC), myxoyd glioneuronal tumour (MGT) and multinodular and vacuolating neuronal tumour (MNVNT) are new types. MGT and MNVNT are classified WHO grade 1 and may be recognised and diagnosed by peculiar clinical-pathological features. DGONC was not assigned a WHO grade and was only provisionally included among GNT, due to the possibility that it rather represents an embryonal tumour type or subtype. Although the histopathological characteristics may be useful for its identification, the specific methylation profile is an essential diagnostic criterion for DGONC.