Spinal diffuse midline glioma: An unusual case presentation

Primary spinal cord neoplasms are rare, accounting for 1% of paediatric central nervous system tumours. The majority of which are low-grade gliomas (LGG). A teenage male presented with rapidly progressive lower limb paralysis. He subsequently underwent a near-total resection of an intramedullary thoracic tumour. Although the initial histopathology report suggested a LGG, closer review of the morphology and immunohistochemical staining revealed a diffusely infiltrating high-grade astrocytoma. Methylation profiling later confirmed a H3K27M-mutated spinal DMG. He underwent spinal radiation to the tumour bed soon after surgery but succumbed to his disease 8 days after completing radiation, and 3 months after presentation.Conclusion: Spinal DMGs are rare, can lead to diagnostic challenges and have a poor prognosis.

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Publication Details

Journal
South African Journal of Oncology
Published
2024-10-11
DOI
https://doi.org/10.4102/sajo.v8i0.306
Primary Topic
Glioma Diagnosis and Treatment
Type
article
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article

Spinal diffuse midline glioma: An unusual case presentation

Duvern Ramiah, Nadia Beringer, Despina Demopoulos, Kate G. Bennett et al.
South African Journal of Oncology
Glioma Diagnosis and Treatment
article

Spinal diffuse midline glioma: An unusual case presentation

Duvern Ramiah, Nadia Beringer, Despina Demopoulos, Kate G. Bennett, Raazik Gani, Christos Profyris, Abhijit Joshi, L Doedens, John Ouma, Marelize Reynders, Deborah Pearce, Dawn-Lee Van der Byl, Simon Bailey, Pieter E. Boshoff, Felicia Tshite
article en

Abstract

Primary spinal cord neoplasms are rare, accounting for 1% of paediatric central nervous system tumours. The majority of which are low-grade gliomas (LGG). A teenage male presented with rapidly progressive lower limb paralysis. He subsequently underwent a near-total resection of an intramedullary thoracic tumour. Although the initial histopathology report suggested a LGG, closer review of the morphology and immunohistochemical staining revealed a diffusely infiltrating high-grade astrocytoma. Methylation profiling later confirmed a H3K27M-mutated spinal DMG. He underwent spinal radiation to the tumour bed soon after surgery but succumbed to his disease 8 days after completing radiation, and 3 months after presentation.Conclusion: Spinal DMGs are rare, can lead to diagnostic challenges and have a poor prognosis.

South African Journal of OncologyVol. 8
University of the Witwatersrand (ZA), Milpark Hospital (ZA), Wits University Donald Gordon Medical Centre (ZA), Great North Children's Hospital (GB)
No poverty
Openalex Percentile: Top 70%
Glioma Diagnosis and Treatment
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Spinal diffuse midline glioma: An unusual case presentation — Duvern Ramiah, Nadia Beringer, et al. · South African Journal of Oncology (2024) | TGRS Research Map | TGRS