JNO special issue: an update on pediatric neuro-oncology
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EDITORIAL
JNO special issue: an update on pediatric neuro‑oncology L. B. Kilburn1,2 · Roger J. Packer1,3 Received: 28 May 2020 / Accepted: 13 June 2020 © Springer Science+Business Media, LLC, part of Springer Nature 2020
In spite of tremendous advances in our Queryunderstanding of the biology of many pediatric brain tumors, they remain the leading cause of death due to cancer in children [1]. Significant progress has been made in better stratifying patients based on both clinical and molecular tumor characteristic resulting in improvements in the long term survival, quality of life or late effects in some children with tumors such as medulloblastoma or low grade glioma. However there remain many pediatric brain tumor types for which little or no therapeutic progress has been made over many decades. New therapeutic approaches remain greatly needed. Over the last two decades the critical role of epigenetics in cancer biology has evolved significantly. In parallel our understanding of the biology of many pediatric brain tumors and the central role of alterations in their epigenetic regulation has become an important area of discovery. Hopefully over the next decade the biologic advances, which are detailed throughout this issue, in understanding the role of epigenetics will further characterize pediatric brain tumors, open new therapeutic avenues for treatment and lead to true breakthroughs and cures for children [2]. Research toward understanding the balance between transcriptional activation and repression through chromatin modifications and the role of epigenetic regulation in cancer development, maintenance and resistance to therapy continues to evolve. Starting with the early recognition that cancers had abnormal gain and loss of methylation, through identification of gene mutations in promoter regions or components of histones or chromatin remodeling complexes across cancer types, to more recent tumor specific research into
* Roger J. Packer [email protected] 1
Brain Tumor Institute, Children’s National Hospital, Washington, DC, USA
2
Center for Cancer and Blood Disorders, Children’s National Hospital, Washington, DC, USA
3
Center for Neuroscience and Behavioral Medicine, Children’s National Hospital, Washington, DC, USA
the implications of these changes on tumor phenotype and microenvironment, our increased understanding of the role of epigenetics in cancer addresses unanswered questions but also lead to new areas of research. In contrast to many adult tumors, which demonstrate distinct tumor mutations and higher mutational burden, the paucity of recurrent mutations in pediatric cancers, have often been highlighted [3]. Embryonal tumors like medulloblastoma, atypical teratoid rhabdoid tumor had long been recognized as biologically distinct from brain tumors which occur more commonly in adults and tumor clustering and categorization by genome-wide DNA methylation and gene expression analysis has further substantiated these differences. More recent discoveries of mutations in histone genes in pe
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