Diffuse glioneuronal tumor with oligodendroglial features and nuclear clusters is a glioneuronal tumor entity with a characteristic methylation profile 1.
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Terminology
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.
Epidemiology
Diffuse glioneuronal tumor with oligodendroglial features and nuclear clusters occurs mainly in children, although some higher-age cases have been described 1, 3.
Pathology
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 entity based on its specific methylation profile.
Location
It arises in the cerebral hemispheres, preferentially from the temporal lobes 1.
Microscopic appearance
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.
Immunophenotype
Radiographic features
MRI
T1: hypointense
T2/FLAIR: hyperintense with no perilesional edema
T1 C+ (Gd): poor enhancement 3
Treatment and prognosis
Data about the treatment and prognosis of these 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.
Differential diagnosis
Possible imaging differential considerations include: