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종결 Case No. 715 2025-03-01~2025-03-31
출제자 : 이지영 Hit : 480
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Period 2025-03-01~2025-03-31
Diagnosis Diffuse Midline Glioma with H3K27-altered
Gender
  • M
  •  
  • F
Age 37
Clinical information M/37

c/c: Visual field defect (bitemporal hemianopsia) for 1 year

Initially treated as pituitary macroadenoma with hormone tx (cabergoline) .
worsened visual disturbance
Discussion -Diffuse glioma with H3 K27M or H3 K27I mutation
-High-grade infiltrative glioma with predominantly astrocytic differentiation (WHO Grade IV)
-Mostly in children, but can occur in adults
-Poor prognosis
-Location: Midline or paramedian
Brainstem (pons m/c), Thalamus (2nd), Spinal cord, Hypothalamus…

-Pathology
Fusiform enlargement of pons
Mostly astrocytic differentiation with focal necrosis, hemorrhage, microvascular proliferation

-Brainstem Type:
Diffuse infiltration of pons with indistinct margins
Often expands anteriorly, engulfing basilar artery
T1WI: Hypointense
T2WI/FLAIR: Hyperintense
Contrast: No or little patchy enhancement

-Thalamic Type:
Enlarged thalamus
T2/FLAIR: Hyperintense
Variable enhancement, but usually minimal or patchy (<25%)

-Metastatic Pattern: Leptomeningeal dissemination and "brain-to-brain" metastases common in all types



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  • 김상흠 연세대학교 세브란스병원
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