Dysembryoplastic Neuroepithelial Tumors Share with Pleomorphic Xanthoastrocytomas and Gangliogliomas BRAFV600E Mutation and Expression. (20th March 2013)

• Record Type: Journal Article
• Authors: Chappé, Céline; Padovani, Laetitia; Scavarda, Didier; Forest, Fabien; Nanni‐Metellus, Isabelle; Loundou, Anderson; Mercurio, Sandy; Fina, Frédéric; Lena, Gabriel; Colin, Carole; Figarella‐Branger, Dominique
• Is Part Of: Brain pathology. Volume 23:Number 5(2013:Sep.)
• Page Start: 574
• Page End: 583
• Publication Date: 2013-03-20
• Subjects: Nervous system -- Diseases -- Periodicals; Brain -- Diseases -- Periodicals; Neurology -- Periodicals; Brain Diseases -- Periodicals; Cerveau -- Maladies -- Périodiques; Système nerveux -- Maladies -- Périodiques; Neurologie -- Périodiques; 616.805
• DOI: 10.1111/bpa.12048
• Languages: English
• ISSNs: 1015-6305

Abstract

<abstract abstract-type="main"> <title>Abstract</title> <p>Pediatric cortical glioneuronal benign tumors mainly include gangliogliomas (GG) [differential diagnoses pilocytic astrocytomas (PA) and pleomorphic xanthoastrocytomas (PXA)] and dysembryoplastic neuroepithelial tumor (DNT). DNT include the specific form and the controversial non‐specific form that lack the specific glioneuronal element. Our aims were to search for <italic>BRAF<sup>V600E</sup></italic> mutation and CD34 expression in DNT, PXA, GG and PA to correlate <italic>BRAF<sup>V600E</sup></italic> mutation with BRAF<sup>V600E</sup> expression and to evaluate their diagnostic and prognostic values. Ninety‐six children were included. <italic>BRAF<sup>V600E</sup></italic> mutation was studied by sequencing and immunohistochemistry; CD34 expression was analyzed by immunohistochemistry. <italic>BRAF<sup>V600E</sup></italic> mutation was detected in PXA (60%), GG (38.7%), DNT (30%, including 3/11 specific and 3/9 non‐specific forms) and PA (12.5%). BRAF<sup>V600E</sup> expression was recorded in PXA (60%), GG (45.2%) and DNT (30%). CD34 expression was recorded in PXA (60%), GG (58.1%), DNT (25%) and PA (12.5%). Neither CD34 expression nor <italic>BRAF<sup>V600E</sup></italic> status was predictive of prognosis, except for PA tumors where CD34 expression was associated with a shorter overall survival. In conclusion, DNT shared with PXA and GG, <italic>BRAF<sup>V600E</sup></italic> mutation and/or CD34 expression, which represent molecular markers for these tumors, and we recommend searching for CD34 expression and <italic>BRAF<sup>V600E</sup></italic> mutation in all DNT, especially the non‐specific forms.</p> </abstract>