Highly aggressive brain tumor, mostly found in children
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Medical condition
Diffuse midline glioma
Magnetic resonance imaging of a diffuse intrinsic pontine glioma.
Usual onset
5–10 years old[1]
Treatment
Radiation Chemotherapy (Surgery to biopsy or remove the tumor is not safe due to its location)[1]
Prognosis
Average overall survival generally ranges from 8 to 11 months[2]
Frequency
~10–20% of childhood brain tumors[1]
Diffuse midline glioma, H3 K27-altered (DMG) is a fatal 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).[3]
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.[4][5] Currently, the standard of care is fractionated external beam radiotherapy, as the tumour location precludes surgery and chemotherapy has shown to be ineffective.[6][7] However, the estimated survival post-diagnosis remains only 9–15 months.
^ abc"Diffuse Intrinsic Pontine Glioma (DIPG)". St. Jude Children's Research Hospital. Retrieved March 12, 2023.
^"Diffuse midline glioma (DIPG) prognosis". thebraintumourcharity.org. Retrieved March 12, 2023.
^Central Nervous System Tumours (5th ed.). International Agency for Research on Cancer. 2022. pp. 69–73. ISBN 9789283245087.
^Vanan MI, Eisenstat DD (2015). "DIPG in Children - What Can We Learn from the Past?". Frontiers in Oncology. 5: 237. doi:10.3389/fonc.2015.00237. PMC 4617108. PMID 26557503.
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