Details

Autor(en) / Beteiligte

• 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

Titel

Clinical, Imaging, Histopathological and Molecular Characterization of Anaplastic Ganglioglioma

Ist Teil von

• Journal of neuropathology and experimental neurology, 2016-10, Vol.75 (10), p.971-980

Ort / Verlag

England: Oxford University Press

Erscheinungsjahr

2016

Quelle

Oxford Journals 2020 Medicine

Beschreibungen/Notizen

• 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.