Diffuse midline glioma with H3 K27M mutation

Diffuse midline glioma with H3 K27M mutation is an aggressive, high-grade malignancy of the central nervous system that arises predominantly in children and young adults, though it can occur at any age Pathophysiologically, the disease is defined by a recurrent somatic missense mutation substituting methionine for lysine at position 27 in histone H3 variants (such as H3.3 or H3.1), which causes global epigenetic dysregulation via the loss of repressive histone H3 lysine 27 trimethylation and dri...

Diffuse midline glioma with H3 K27M mutation is an aggressive, high-grade malignancy of the central nervous system that arises predominantly in children and young adults, though it can occur at any age

Classified as a World Health Organization grade 4 neoplasm, it infiltrates critical midline neuroaxis structures, most commonly the brainstem—historically recognized as diffuse intrinsic pontine glioma—as well as the thalamus and spinal cord

Classified as a World Health Organization grade 4 neoplasm, it infiltrates critical midline neuroaxis structures, most commonly the brainstem—historically recognized as diffuse intrinsic pontine glioma—as well as the thalamus and spinal cord

Classified as a World Health Organization grade 4 neoplasm, it infiltrates critical midline neuroaxis structures, most commonly the brainstem—historically recognized as diffuse intrinsic pontine glioma—as well as the thalamus and spinal cord

Pathophysiologically, the disease is defined by a recurrent somatic missense mutation substituting methionine for lysine at position 27 in histone H3 variants (such as H3.3 or H3.1), which causes global epigenetic dysregulation via the loss of repressive histone H3 lysine 27 trimethylation and drives rapid tumor progression with a historically poor prognosis