title: BRAF(V600E) mutation is a negative prognosticator in pediatric ganglioglioma.
abstract: Gangliogliomas are typically low-grade neuroepithelial tumors seen in the pediatric and young adult populations. Despite their often bland histologic appearance, these tumors recur with varying frequencies; however, little data exist that adequately predict ganglioglioma recurrence in children. To identify potential histopathologic features predictive of recurrence-free survival, a series of 53 patients with World Health Organization (WHO) grade I gangliogliomas were evaluated, representing the largest cohort of pediatric gangliogliomas with accompanying histopathologic and survival data. Fifteen patients (28 %) exhibited disease recurrence during the study period. BRAF(V600E) immunohistochemistry was performed on 47 of these tumors. Histopathologic features associated with shorter recurrence-free survival included an absence of oligodendroglial morphology, higher glial cell density, microvascular proliferation, and the presence of a high lymphoplasmacytic inflammatory infiltrate. Eighteen tumors (38.3 %) had positive BRAF(V600E) staining, which was associated with shorter recurrence-free survival. Collectively, the combined use of histopathologic and molecular features to stratify grade I gangliogliomas into low and high-risk groups provides important information relevant to the management of children and young adults with these rare tumors.
authors.affiliation: Department of Pathology and Immunology, Washington University School of Medicine, St. Louis, MO, USA.
authors.forename: Sonika
authors.initials: S
authors.lastname: Dahiya
authors.forename: Devon H
authors.initials: DH
authors.lastname: Haydon
authors.forename: David
authors.initials: D
authors.lastname: Alvarado
authors.forename: Christina A
authors.initials: CA
authors.lastname: Gurnett
authors.forename: David H
authors.initials: DH
authors.lastname: Gutmann
authors.forename: Jeffrey R
authors.initials: JR
authors.lastname: Leonard
mesh_terms: D000293:Adolescent; D001932:Brain Neoplasms / Q000378:metabolism* / Q000401:mortality / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D018572:Disease-Free Survival; D005260:Female; D018303:Ganglioglioma / Q000378:metabolism* / Q000401:mortality / Q000473:pathology*; D006801:Humans; D007223:Infant; D008297:Male; D050505:Mutant Proteins / Q000378:metabolism*; D048493:Proto-Oncogene Proteins B-raf / Q000378:metabolism*; D015996:Survival Rate; D055815:Young Adult
chemical_list: D050505:Mutant Proteins; C482119:BRAF protein, human; D048493:Proto-Oncogene Proteins B-raf