Diffuse glioneuronal tumor with oligodendroglioma like features and nuclear clusters

Last revised by Dr Francesco Buemi on 25 Nov 2021

Diffuse glioneuronal tumor with oligodendroglial features and nuclear clusters is a novel glioneuronal tumor entity recently identified by a characteristic methylation profile 1.

The exact nature of this tumor has yet to be well defined. In fact, its histological features almost overlap with other better-known tumors such as oligodendroglioma grade 3 and primitive neuroectodermal tumors 1.

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

It occurs mainly in children, although some higher-age cases have been described 1, 3.

This tumor demonstrates histological, molecular and immunophenotype features reminiscent of a wide spectrum of tumors, such as oligodendroglioma grade 3, neurocytoma,  dysembryoplastic neuroepithelial tumor (DNET)primitive neuroectodermal tumors and even glioblastoma 1, 3. Therefore it is likely that in the next years many previously diagnosed high-grade CNS tumors will be assigned to this new entity based on its specific methylation profile

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

This tumor demonstrates clear cell features with moderate to high cellularity and diffuse infiltration, round nuclei with oligodendroglioma-like perinuclear haloes, nuclear clusters and vascular proliferation 1.

  • T1: hypointense
  • T2/FLAIR : hyperintense with no peri-lesional edema
  • T1 C+ (Gd): poor enhancement 3

Data about treatment and prognosis 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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