Diffuse midline glioma, H3 K27M-mutant (DMG)
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
- 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.
- Vuong, Huy Gia, et al. "Prognostic implication of patient age in H3K27M-mutant midline gliomas." Frontiers in Oncology 12 (2022): 858148.
- 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 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.
Delete lesion
Do you really want to delete lesion Diffuse midline glioma?