{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,21]],"date-time":"2026-10-21T15:07:37Z","timestamp":1792595257315,"version":"4.5.0"},"reference-count":110,"publisher":"Oxford University Press (OUP)","issue":"1","license":[{"start":{"date-parts":[[2023,11,7]],"date-time":"2023-11-07T00:00:00Z","timestamp":1699315200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2024,1,5]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>The most common childhood central nervous system (CNS) tumor is pediatric low-grade glioma (pLGG), representing 30%\u201340% of all CNS tumors in children. Although there is high associated morbidity, tumor-related mortality is relatively rare. pLGG is now conceptualized as a chronic disease, underscoring the importance of functional outcomes and quality-of-life measures. A wealth of data has emerged about these tumors, including a better understanding of their natural history and their molecular drivers, paving the way for the use of targeted inhibitors. While these treatments have heralded tremendous promise, challenges remain about how to best optimize their use, and the long-term toxicities associated with these inhibitors remain unknown. The International Pediatric Low-Grade Glioma Coalition (iPLGGc) is a global group of physicians and scientists with expertise in pLGG focused on addressing key pLGG issues. Here, the iPLGGc provides an overview of the current state-of-the-art in pLGG, including epidemiology, histology, molecular landscape, treatment paradigms, survival outcomes, functional outcomes, imaging response, and ongoing challenges. This paper also serves as an introduction to 3 other pLGG manuscripts on (1) pLGG preclinical models, (2) consensus framework for conducting early-phase clinical trials in pLGG, and (3) pLGG resistance, rebound, and recurrence.<\/jats:p>","DOI":"10.1093\/neuonc\/noad195","type":"journal-article","created":{"date-parts":[[2023,11,9]],"date-time":"2023-11-09T19:14:45Z","timestamp":1699557285000},"page":"25-37","source":"Crossref","is-referenced-by-count":95,"title":["Pediatric low-grade glioma: State-of-the-art and ongoing challenges"],"prefix":"10.1093","volume":"26","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3099-5259","authenticated-orcid":false,"given":"Jason","family":"Fangusaro","sequence":"first","affiliation":[{"name":"Department of Hematology and Oncology, Children\u2019s Healthcare of Atlanta and Emory University , Atlanta, Georgia , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David T","family":"Jones","sequence":"additional","affiliation":[{"name":"Translational Program, Hopp Children\u2019s Cancer Center Heidelberg (KiTZ) , Heidelberg , Germany"},{"name":"German Cancer Research Center (DKFZ), University Hospital Heidelberg , Heidelberg , Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roger J","family":"Packer","sequence":"additional","affiliation":[{"name":"Brain Tumor Institute, Daniel and Jennifer Gilbert Neurofibromatosis Institute, Neuroscience and Behavioral Medicine, Children\u2019s National Medical Center , Washington, District of Columbia , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David H","family":"Gutmann","sequence":"additional","affiliation":[{"name":"Department of Neurology, Washington University School of Medicine , St. Louis, Missouri , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7267-1052","authenticated-orcid":false,"given":"Till","family":"Milde","sequence":"additional","affiliation":[{"name":"Translational Program, Hopp Children\u2019s Cancer Center Heidelberg (KiTZ) , Heidelberg , Germany"},{"name":"German Cancer Research Center (DKFZ), University Hospital Heidelberg , Heidelberg , Germany"},{"name":"Clinical Cooperation Unit Pediatric Oncology, German Cancer Research Center (DKFZ) and German Consortium for Translational Cancer Research (DKTK) , Heidelberg , Germany"},{"name":"KiTZ Clinical Trial Unit (ZIPO), Department of Pediatric Hematology, Oncology, Immunology and Pulmonology, Heidelberg University Hospital; National Center for Tumor Diseases (NCT) , Heidelberg , Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Olaf","family":"Witt","sequence":"additional","affiliation":[{"name":"Translational Program, Hopp Children\u2019s Cancer Center Heidelberg (KiTZ) , Heidelberg , Germany"},{"name":"German Cancer Research Center (DKFZ), University Hospital Heidelberg , Heidelberg , Germany"},{"name":"Clinical Cooperation Unit Pediatric Oncology, German Cancer Research Center (DKFZ) and German Consortium for Translational Cancer Research (DKTK) , Heidelberg , Germany"},{"name":"KiTZ Clinical Trial Unit (ZIPO), Department of Pediatric Hematology, Oncology, Immunology and Pulmonology, Heidelberg University Hospital; National Center for Tumor Diseases (NCT) , Heidelberg , Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sabine","family":"Mueller","sequence":"additional","affiliation":[{"name":"Department of Neurological Surgery, University of California , San Francisco, California , USA"},{"name":"Department of Pediatrics, University of California , San Francisco, California , USA"},{"name":"Department of Neurology, University of California , San Francisco, California , USA"},{"name":"Department of Oncology, University Children\u2019s Hospital Z\u00fcrich , Z\u00fcrich , Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael J","family":"Fisher","sequence":"additional","affiliation":[{"name":"Division of Oncology, The Children\u2019s Hospital of Philadelphia , Philadelphia, Pennsylvania , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jordan R","family":"Hansford","sequence":"additional","affiliation":[{"name":"Michael Rice Centre for Hematology and Oncology, Women\u2019s and Children\u2019s Hospital , Adelaide, South Australia , Australia"},{"name":"South Australia Health and Medical Research Institute , Adelaide, South Australia , Australia"},{"name":"South Australia ImmunoGENomics Cancer Institute, University of Adelaide , Adelaide, South Australia , Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5019-2683","authenticated-orcid":false,"given":"Uri","family":"Tabori","sequence":"additional","affiliation":[{"name":"The Hospital for Sick Children, University of Toronto , Toronto, Ontario , Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Darren","family":"Hargrave","sequence":"additional","affiliation":[{"name":"University College London Great Ormond Street Institute of Child Health , London , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4175-4760","authenticated-orcid":false,"given":"Pratiti","family":"Bandopadhayay","sequence":"additional","affiliation":[{"name":"Department of Pediatric Oncology, Dana-Farber\/Boston Children\u2019s Cancer and Blood Disorders Center , Boston, Massachusetts , 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