Children are not just little adults: Recent advances in understanding of diffuse intrinsic pontine glioma biology

Pediatric Research, 01/08/2014

Diffuse intrinsic pontine glioma (DIPG) is a high–grade glioma that originates in the pons and is seen exclusively in children. Despite numerous efforts to improve treatment, DIPG remains incurable with 90% of children dying within 2 y of diagnosis, making it one of the leading causes of death in children with brain tumors. With the advent of new genomic tools, the genetic landscape of DIPG is slowly being unraveled. The most common genetic alterations include a K27M mutation in H3.3 or H3.1, which are found in up to 78% of DIPGs, whereas p53 mutations are found in up to 77%.

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