Diffuse glioneuronal tumor with oligodendroglioma like features and nuclear clusters

Last revised by David Luong on 07 Jan 2022

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 diffuse glioneuronal tumors with oligodendroglial features and nuclear clusters 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

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

Diffuse glioneuronal tumor with oligodendroglial features and nuclear clusters 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 near future many previously diagnosed high-grade CNS tumors will be re-assigned to this new entity based on its specific methylation profile.

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

Diffuse glioneuronal tumor 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 edema
  • T1 C+ (Gd): poor enhancement 3

Data about the treatment and prognosis of these newly characterized tumors 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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