Glioblastoma with primitive neuronal components
Citation, DOI & case data
1 month of progressive expressive and nominal dysphasia, confusion, memory loss.
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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.
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.
- Poyuran R, Chandrasekharan K, Easwer HV, Narasimhaiah D. Glioblastoma with primitive neuronal component: An immunohistochemical study and review of literature. Journal of Clinical Neuroscience. 2021;93:130-136. ISSN 0967-5868, https://doi.org/10.1016/j.jocn.2021.09.004.
- Rajalakshmi Poyuran, Kesavadas Chandrasekharan, H.V. Easwer, Deepti Narasimhaiah, Glioblastoma with primitive neuronal component: An immunohistochemical study and review of literature, Journal of Clinical Neuroscience, Volume 93, 2021, Pages 130-136, ISSN 0967-5868, https://doi.org/10.1016/j.jocn.2021.09.004.
- Shayganfar A, Ebrahimian S, Mahzouni P, Shirani F, Aalinezhad M. A review of glioblastoma tumors with primitive neuronal component and a case report of a patient with this uncommon tumor at a rare location. Clinical Case Reports. 2020 Aug 12;8:12. doi: 10.1002/ccr3.3228.
- Sánchez-Ortega JF, Aguas-Valiente J, Sota-Ochoa P, Calatayud-Pérez J. Glioblastoma with primitive neuronal component: A case report and considerations of fluorescence-guided surgery. Surg Neurol Int. 2020 Jul 4;11:178. doi: 10.25259/SNI_272_2020. PMID: 32754353; PMCID: PMC7395537.