Glioblastoma with primitive neuronal components

Case contributed by Siobhan Lee


1 month of progressive expressive and nominal dysphasia, confusion, memory loss.

Patient Data

Age: 70 years
Gender: Male

Large cystic mass in the left temporal lobe with peripheral enhancement, with minor anterolateral nodularity, but no large solid component. Moderate vasogenic edema and mass effect, with 7mm of midline shift, ipsilateral lateral ventricle narrowing with contralateral dilatation, and early uncal herniation. No cerebellar tonsillar herniation.

No other intracranial lesions.

No evidence of dural or leptomeningeal enhancement.

Case Discussion

The patient presented to the emergency department at a regional hospital. On initial CT, the differential diagnoses suggested included glioma/astrocytoma and gangliogloma. MRI was not organized initially due to concerns about pacemaker compatibility. The patient was transferred to a tertiary center and underwent resection.

Biopsy demonstrated glioblastoma, IDH-wildtype, with primitive neuronal component, MGMT promoter methlyated (average CpG site methylation rate 21%). The tumor was negative for 1p/19q co-deletion, EGFR amplification, IDH1/IDH2, BRAF, H3F3A mutations.

Glioblastoma with primitive neuronal components are a rare subtype of glioblastoma (~0.5%), with components arising from the primitive neuroectoderm. They are associated with a more aggressive course, and more prone to CSF dissemination.

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