Diffuse glioneuronal tumour with oligodendroglioma like features and nuclear clusters

Last revised by Rohit Sharma on 5 Apr 2024

Diffuse glioneuronal tumour with oligodendroglial features and nuclear clusters is a glioneuronal tumour entity with a characteristic methylation profile 1.

The exact nature of diffuse glioneuronal tumours with oligodendroglial features and nuclear clusters has yet to be well defined. In fact, its histological features almost overlap with other better-known tumours such as oligodendroglioma grade 3 and primitive neuroectodermal tumours 1.

It has been provisionally included in the 5th Edition (2021) WHO brain tumour classification 2, but more studies are needed for full acceptance. It has been proposed it should be considered a WHO grade 2 tumour 1

Diffuse glioneuronal tumour with oligodendroglial features and nuclear clusters occurs mainly in children, although some higher-age cases have been described 1, 3.

Diffuse glioneuronal tumour with oligodendroglial features and nuclear clusters demonstrates histological, molecular and immunophenotype features reminiscent of a wide spectrum of tumours, such as oligodendroglioma grade 3, neurocytoma,  dysembryoplastic neuroepithelial tumour (DNET)primitive neuroectodermal tumours and even glioblastoma 1, 3. Therefore it is likely that in the near future many previously diagnosed high-grade CNS tumours will be re-assigned to this entity based on its specific methylation profile.

It arises in the cerebral hemispheres, preferentially from the temporal lobes 1.

Diffuse glioneuronal tumour with oligodendroglial features and nuclear clusters demonstrates clear cell features with moderate to high cellularity and diffuse infiltration, round nuclei with oligodendroglioma-like perinuclear halos, nuclear clusters and vascular proliferation 1.

  • T1: hypointense

  • T2/FLAIR: hyperintense with no perilesional oedema

  • T1 C+ (Gd): poor enhancement 3

Data about the treatment and prognosis of these tumours are still limited. In the few reported cases 1,3, surgical resection was the treatment of choice with adjuvant radiation therapy and chemotherapy. Prognosis is generally positive although recurrence is possible.

Possible imaging differential considerations include:

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