Diffuse midline glioma, H3 K27M-mutant (DMG)

Gliomas

General description

Diffuse midline glioma, H3 K27M-mutant (DMG) represents a distinct tumor entity that exhibits heterogeneous epidemiological patterns across age groups. The age distribution shows a bimodal pattern, with the median age at diagnosis being approximately 9 years in pediatric patients and 33 years in adult patients. While historically considered predominantly pediatric tumors, recent cohort studies demonstrate that adult cases comprise 57.3% of all H3 K27M-mutant DMGs, with 34.8% of patients being over 30 years of age. The overall male-to-female ratio is approximately 1.2:1, though this varies by age group, with adult patients showing a slight male predominance while pediatric patients demonstrate a slight female predominance. The oldest reported patient with H3 K27M-mutant DMG was 81 years of age.

References

  1. Zheng, Linmao, et al. "Diffuse midline gliomas with histone H3 K27M mutation in adults and children: a retrospective series of 164 cases." The American Journal of Surgical Pathology 46.6 (2022): 863-871.
  2. Vuong, Huy Gia, et al. "Prognostic implication of patient age in H3K27M-mutant midline gliomas." Frontiers in Oncology 12 (2022): 858148.
  3. Schulte, Jessica D., et al. "Clinical, radiologic, and genetic characteristics of histone H3 K27M-mutant diffuse midline gliomas in adults." Neuro-oncology advances 2.1 (2020): vdaa142.

Diffuse midline glioma

Anatomical regions
  • Brainstem
  • Thalamus
  • Ventricle
  • Caudate nucleus
  • Putamen
  • Globus pallidus
  • Cerebellum
  • Spinal cord
Asymmetric
Midline
Medial
Morphology
Enlargement / swelling
Ill-defined border
Plain CT
Normal attenuation
Low attenuation
T1WI
Hypointensity
T2WI
Hyperintensity
FLAIR
Hyperintensity
DWI
Hyperintensity
ADC
Hypointensity

Anatomical location preferences differ significantly between age groups. In pediatric patients, the most frequent location is the brainstem (62.9% of cases), whereas in adults, thalamic involvement predominates (35.1% of cases). The tumors arise from midline structures of the central nervous system including the thalamus (38-46% of cases), brainstem (33-75% of cases), spinal cord (4.3-12.5% of cases), ventricular system, basal ganglia, pineal region, cerebellum, and sellar region. Despite their midline predilection, H3 K27M-mutant tumors can occasionally arise in non-midline hemispheric locations, though these represent rare occurrences.