title: Advances in the biology and treatment of pediatric central nervous system tumors.
abstract: OBJECTIVE
  Central nervous system tumors represent the most common solid tumors in children and are a leading cause of cancer-related fatalities in this age group. Here, we provide an update on insights gained through molecular profiling of the most common malignant childhood brain tumors.
  
  
  RESULTS
  Genomic profiling studies of medulloblastoma, ependymoma, and diffuse intrinsic pontine glioma (diffuse midline glioma, with H3-K27M mutation), have refined, if not redefined, the diagnostic classification and therapeutic stratification of patients with these tumors. They detail the substantial genetic heterogeneity across each disease type and, importantly, link genotypic information to clinical course. The most aggressive, treatment-resistant (and also treatment-sensitive) forms within each disease entity are identified, and their potentially actionable targets.
  
  
  CONCLUSIONS
  Molecularly based classification of pediatric brain tumors provides a critical framework for the more precise stratification and treatment of children with brain tumors.
authors.affiliation: aDepartment of Neurology & Neurological Sciences bDepartment of Neurosurgery, Stanford University School of Medicine, Stanford, California, USA.
authors.forename: Nicholas A
authors.initials: NA
authors.lastname: Vitanza
authors.forename: Yoon-Jae
authors.initials: YJ
authors.lastname: Cho
mesh_terms: D016543:Central Nervous System Neoplasms / Q000235:genetics* / Q000628:therapy*; D002528:Cerebellar Neoplasms / Q000235:genetics / Q000628:therapy; D002648:Child; D004806:Ependymoma / Q000235:genetics / Q000628:therapy; D020869:Gene Expression Profiling; D020022:Genetic Predisposition to Disease; D005910:Glioma / Q000235:genetics / Q000628:therapy; D006801:Humans; D008527:Medulloblastoma / Q000235:genetics / Q000628:therapy; D009154:Mutation