title: New strategies in pediatric gliomas: molecular advances in pediatric low-grade gliomas as a model.
abstract: Pediatric low-grade gliomas (pLGG) account for more brain tumors in children than any other histologic subtype. While surgery, chemotherapy and radiation remain the mainstay of upfront treatment, recent advances in molecular interrogation of pLGG have shown a small number of recurring genetic mutations in these tumors that might be exploited therapeutically. Notable findings include abnormalities in the RAS/MAP kinase pathway such as NF-1 loss or BRAF activation and mTOR activation. Recent identification of activating re-arrangements in c-MYB and MYBL1 in pediatric diffuse astrocytoma also provide candidates for therapeutic intervention. Targeting these molecularly identified pathways may allow for improved outcomes for patients as pediatric oncology moves into the era of biology-driven medicine.
authors.affiliation: The Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins, Baltimore, Maryland 21287, USA.
authors.forename: Eric
authors.initials: E
authors.lastname: Raabe
authors.forename: Mark W
authors.initials: MW
authors.lastname: Kieran
authors.forename: Kenneth J
authors.initials: KJ
authors.lastname: Cohen
mesh_terms: D001932:Brain Neoplasms / Q000145:classification / Q000235:genetics* / Q000473:pathology / Q000628:therapy*; D002648:Child; D005910:Glioma / Q000145:classification / Q000235:genetics* / Q000473:pathology / Q000628:therapy*; D006801:Humans; D058990:Molecular Targeted Therapy; D009154:Mutation; D060787:Neoplasm Grading; D009364:Neoplasm Recurrence, Local / Q000235:genetics / Q000473:pathology / Q000628:therapy; D025542:Neurofibromin 1 / Q000235:genetics; D010372:Pediatrics / Q000639:trends; D011518:Proto-Oncogene Proteins / Q000235:genetics; D048493:Proto-Oncogene Proteins B-raf / Q000235:genetics; D020598:Proto-Oncogene Proteins c-myb / Q000235:genetics; D058570:TOR Serine-Threonine Kinases / Q000235:genetics; D015534:Trans-Activators / Q000235:genetics
chemical_list: C088831:MYBL1 protein, human; D025542:Neurofibromin 1; D011518:Proto-Oncogene Proteins; D020598:Proto-Oncogene Proteins c-myb; D015534:Trans-Activators; C546842:MTOR protein, human; C482119:BRAF protein, human; D048493:Proto-Oncogene Proteins B-raf; D058570:TOR Serine-Threonine Kinases