HEMATOLOGY AND ONCOLOGY: Edited by Susan M. BlaneyManagement of pediatric low-grade gliomade Blank, Petera,b; Bandopadhayay, Pratitic,d,e; Haas-Kogan, Daphned,f; Fouladi, Maryama,b; Fangusaro, Jasong,hAuthor Information aUniversity of Cincinnati bDivision of Oncology, Cancer and Blood Diseases Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio cDepartment of Pediatrics, Dana-Farber/Boston Children's Cancer and Blood Disorders Center dHarvard Medical School eBroad Institute of MIT and Harvard, Boston, Massachusetts fDepartment of Radiation Oncology, Brigham and Women's Hospital, Dana-Farber Cancer Institute, Boston Children's Hospital gDivision of Oncology, Aflac Cancer and Blood Disorders Center, Children's Healthcare of Atlanta hEmory University School of Medicine, Atlanta, Georgia, USA Correspondence to Peter de Blank, MD, MSCE, Cincinnati Children's Hospital Medical Center, 3333 Burnet Avenue, MLC 7018, Cincinnati, OH 45229-3026, USA. Tel: +1 513 517 2068; fax: +1 513 636 3549; e-mail: [email protected] Current Opinion in Pediatrics: February 2019 - Volume 31 - Issue 1 - p 21-27 doi: 10.1097/MOP.0000000000000717 Buy Metrics Abstract Purpose of review Pediatric low-grade gliomas (pLGGs) have been treated with similar therapies for the last 30 years. Recent biological insights have allowed a new generation of targeted therapies to be developed for these diverse tumors. At the same time, technological advances may redefine the late toxicities associated with radiation therapy. Understanding recent developments in pLGG therapy is essential to the management of these common pediatric tumors. Recent findings It is now well understood that aberrations of the mitogen-activated protein kinase pathway are key to oncogenesis in low-grade gliomas. This understanding, along with the development of available targeted agents, have heralded a new era of understanding and treatment for these patients. Promising, sustained responses are now being seen in early phase trials among patients with multiply recurrent/progressive disease. Also, newer and highly conformal radiation approaches such as proton beam radiotherapy maintain efficacy of radiation but limit radiation-associated toxicities. Summary Novel therapies offer the potential for tumor control with greatly reduced toxicities. However, late effects of these therapies are just now being explored. Improved radiation approaches and targeted agents have the potential to redefine traditional therapy for pLGG. Copyright © 2018 Wolters Kluwer Health, Inc. All rights reserved.