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H3K27M-mutant spinal cord and hemispheric tumor with prolonged survival: illustrative case
Eva Liu1, Hsuan Ming Su1, Patrick Toyota1
1Division of Neurosurgery, University of Saskatchewan, Saskatoon, Saskatchewan, Canada.
Background:
H3K27M-altered diffuse midline glioma (DMG) is a recently classified tumor in the 2021 WHO classification of central nervous system tumors. Pediatric patients with H3K27M-altered DMG have a universally poor prognosis, with a median survival of 9-15 months. The prognosis of adult patients with the same tumor type is more variable, with rare cases of prolonged survival.
Observations:
The authors report the case of a 32-year-old male who presented with Brown-Séquard syndrome secondary to a heterogeneously enhancing intramedullary mass extending from T7 to T10. The lesion was resected, and the patient was diagnosed with an H3K27M-altered DMG. The patient presented again 29 months later with a nonenhancing right-sided temporal mass. This was again determined to be an H3K27M-mutated glioma with a molecular signature similar to the intramedullary mass. He received postoperative radiation therapy after each surgery and continued to survive well at the 42-month follow-up.
Lessons:
The authors report the rare case of an adult-onset H3K27M midline glioma in the spinal cord and subsequent dissemination to the right temporal lobe with prolonged survival. This suggests that H3K27M-altered DMGs are heterogeneous entities with variable prognoses based on location and age. https://thejns.org/doi/10.3171/CASE24668.
Insights
This case study highlights a rare adult-onset H3K27M-altered diffuse midline glioma (DMG) initially found in the spinal cord. The patient experienced prolonged survival after treatment for both primary and disseminated tumors.
Area of Science:
- Neuro-oncology
- Molecular Pathology
- Central Nervous System Tumors
Background:
- Diffuse midline glioma (DMG) altered by H3K27M mutation is a distinct entity in the 2021 WHO CNS tumor classification.
- Pediatric H3K27M-altered DMG typically presents a poor prognosis with a median survival of 9-15 months.
- Adult prognoses for H3K27M-altered DMG are more variable, with occasional long-term survival.
Purpose of the Study:
- To report a rare case of adult-onset H3K27M-altered DMG.
- To describe the clinical course and molecular characteristics of a spinal cord DMG with subsequent dissemination.
- To discuss the implications of this case for understanding DMG heterogeneity and prognosis.
Main Methods:
- Case report of a 32-year-old male with spinal cord and temporal lobe masses.
- Surgical resection of both lesions.
- Histopathological and molecular analysis confirming H3K27M-altered glioma.
- Postoperative radiation therapy.
- Clinical follow-up.
Main Results:
- Diagnosis of H3K27M-altered diffuse midline glioma in the spinal cord (T7-T10).
- Recurrence/dissemination 29 months later as a temporal lobe mass with similar molecular signature.
- Patient received radiation therapy after both resections.
- Prolonged survival with well status at 42-month follow-up.
Conclusions:
- This case represents a rare instance of adult-onset spinal H3K27M-altered DMG with temporal lobe dissemination.
- The findings suggest that H3K27M-altered DMGs can exhibit variable prognoses influenced by tumor location and patient age.
- H3K27M-altered DMGs are heterogeneous, necessitating further investigation into prognostic factors.
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