title: An integrative molecular and genomic analysis of pediatric hemispheric low-grade gliomas: an update.
abstract: Hemispheric low-grade gliomas account for the second most common location in pediatric low-grade gliomas (PLGGs) after the cerebellum. The pathological spectrum includes gangliogliomas, dysembryoplastic neuroepithelial tumors (DNETs), diffuse astrocytomas, pilocytic astrocytomas, and pleomorphic xanthoastrocytomas (PXAs), among others. Clinically, hemispheric PLGGs represent a well-recognized cause of intractable epilepsy in children and adolescents. With an excellent long-term outcome, surgery remains the cornerstone and patients with gross total resection typically do not need any further therapies. The recent literature about hemispheric PLGGs was reviewed to provide an up-to-date overview of the molecular and cell biology of these tumors. Hemispheric PLGGs can harbor multiple alterations involving BRAFV600E, FGFR, NTRK, MYB/MYBL1, IDH, and BRAF-KIAA1549 fusions. However, the clinical significance of most of these alterations is still to be defined. The role of RAS/MAPK mutations and other alterations in hemispheric PLGGs is of interest from diagnostic, prognostic, and therapeutic perspectives. Molecular testing for these tumors should be encouraged, since the findings can have an important impact not only in prognosis but also in therapeutic strategies.
authors.affiliation: Division of Pediatric Neuro-oncology, The Hospital for Sick Children, University of Toronto, 555 University Avenue, Toronto, M5G 1X8, ON, Canada.
authors.forename: Alvaro
authors.identifier: http://orcid.org/0000-0003-2881-1473
authors.initials: A
authors.lastname: Lassaletta
authors.affiliation: Division of Pediatric Neuro-oncology, The Hospital for Sick Children, University of Toronto, 555 University Avenue, Toronto, M5G 1X8, ON, Canada.
authors.forename: Michal
authors.initials: M
authors.lastname: Zapotocky
authors.affiliation: Division of Pediatric Neuro-oncology, The Hospital for Sick Children, University of Toronto, 555 University Avenue, Toronto, M5G 1X8, ON, Canada.
authors.forename: Eric
authors.initials: E
authors.lastname: Bouffet
authors.affiliation: Division of Pathology, The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada.
authors.forename: Cynthia
authors.initials: C
authors.lastname: Hawkins
authors.affiliation: Division of Pediatric Neuro-oncology, The Hospital for Sick Children, University of Toronto, 555 University Avenue, Toronto, M5G 1X8, ON, Canada. uri.tabori@sickkids.ca.
authors.forename: Uri
authors.initials: U
authors.lastname: Tabori
mesh_terms: D000293:Adolescent; D000077548:Anaplastic Lymphoma Kinase; D001932:Brain Neoplasms / Q000235:genetics*; D002648:Child; D004268:DNA-Binding Proteins; D005260:Female; D007839:Functional Laterality / Q000235:genetics*; D023281:Genomics / Q000379:methods*; D005910:Glioma / Q000235:genetics*; D006801:Humans; D007521:Isocitrate Dehydrogenase / Q000235:genetics; D008297:Male; D020929:Mitogen-Activated Protein Kinase Kinases / Q000235:genetics; D009154:Mutation / Q000235:genetics; D025542:Neurofibromin 1 / Q000235:genetics; D009687:Nuclear Proteins / Q000235:genetics; D029543:Nucleocytoplasmic Transport Proteins / Q000235:genetics; D048493:Proto-Oncogene Proteins B-raf / Q000235:genetics; D016601:RNA-Binding Proteins; D020794:Receptor Protein-Tyrosine Kinases / Q000235:genetics; D051496:Receptor, Fibroblast Growth Factor, Type 1 / Q000235:genetics; D014157:Transcription Factors; D018631:ras Proteins / Q000235:genetics
chemical_list: D004268:DNA-Binding Proteins; C491719:MYBBP1A protein, human; D025542:Neurofibromin 1; D009687:Nuclear Proteins; D029543:Nucleocytoplasmic Transport Proteins; D016601:RNA-Binding Proteins; D014157:Transcription Factors; D007521:Isocitrate Dehydrogenase; D000077548:Anaplastic Lymphoma Kinase; C496348:FGFR1 protein, human; D020794:Receptor Protein-Tyrosine Kinases; D051496:Receptor, Fibroblast Growth Factor, Type 1; C482119:BRAF protein, human; D048493:Proto-Oncogene Proteins B-raf; D020929:Mitogen-Activated Protein Kinase Kinases; D018631:ras Proteins
keywords: BRAF; FGFR; Hemispheric; Low-grade gliomas; MYB; Pediatric