Diffuse midline glioma

EN Texto en inglés Aún no hay artículo en tu idioma: extracto en inglés.

Diffuse midline glioma, H3 K27-altered (DMG) is a tumour that arises in midline structures of the brain, most commonly the brainstem, thalamus and spinal cord. When located in the pons it is also known as diffuse intrinsic pontine glioma (DIPG). DMG is believed to be caused by genetic mutations that cause epigenetic changes in cells of the developing nervous system, resulting in a failure of the cells to properly differentiate. Currently, the standard of care is fractionated external beam radiotherapy, as the tumour location precludes surgery, and chemotherapy has shown to be ineffective. However, the estimated survival post-diagnosis remains only 9–15 months. DMGs primarily affect children: the median age of diagnosis is around 6-7 years old. Current understanding has shown several genes are involved in the pathology of the glioma. The pathology is resistant to treatment, suggesting that a major driver is that cellular apoptosis mechanisms are disabled.