Clinical, Imaging, Histopathological and Molecular Characterization of Anaplastic Ganglioglioma. Issue 10 (October 2016)

• Record Type: Journal Article
• Authors: Zanello, Marc; Pages, Mélanie; Tauziède-Espariat, Arnault; Saffroy, Raphael; Puget, Stéphanie; Lacroix, Ludovic; Dezamis, Edouard; Devaux, Bertrand; Chrétien, Fabrice; Andreiuolo, Felipe; Sainte-Rose, Christian; Zerah, Michel; Dhermain, Frédéric; Dumont, Sarah; Louvel, Guillaume; Meder, Jean-François; Grill, Jacques; Dufour, Christelle; Pallud, Johan; Varlet, Pascale
• Is Part Of: Journal of neuropathology and experimental neurology. Volume 75:Issue 10(2016)
• Publication Date: 2016-10
• Subjects: Anaplasia -- ATRX -- BRAF -- Ganglioglioma -- H3K27M -- Molecular genetics -- Neoplasm -- Prognosis.; Neurology -- Diseases -- Periodicals; Neurology -- Diseases -- Physiopathology -- Periodicals; 616.8047
• DOI: 10.1093/jnen/nlw074
• Languages: English
• ISSNs: 0022-3069

Abstract

Abstract : Anaplastic ganglioglioma (AGG) is a rare and malignant variant of ganglioglioma. According to the World Health Organization classification version 2016, their histopathological grading criteria are still ill-defined. The aim of the present study was to assess the clinical, imaging, histopathological, and molecular characteristics and outcomes of AGGs in a large consecutive and retrospective adult and pediatric case series. Eighteen patients with AGGs (13 adults and 5 children) were identified (14 de novo and 4 secondary) from a cohort of 222 gangliogliomas (GG) (8%) treated at our institution between 2000 and 2015. AGGs represented a very aggressive disease with poor outcome (median progression-free survival, 10 months; median overall survival, 27 months). They were located in the temporal lobe only in 22% and presented with seizures (44%) or increased intracranial pressure (44%) at diagnosis. Concerning histopathological and molecular data, they shared morphological characteristics and BRAF V600E mutation (39%) with their benign counterparts but also showed hTERT promoter mutation (61%), p53 accumulation (39%), ATRX loss (17%), or p.K27M H3F3A mutation (17%). AGGs are malignant neoplasms requiring aggressive oncological treatment. In the perspective of targeted therapies, AGGs should be screened for BRAF V600E, hTERT, ATRX, and mutations of histone genes.