• Aleix Rosselló, Gerard Plans, Noemi Vidal-Sarró, Alejandro Fernández-Coello, and Andreu Gabarrós.
• Department of Neurosurgery, Hospital Universitari de Bellvitge, l'Hospitalet de Llobregat, Barcelona, Catalonia, Spain. Electronic address: a.rossello@bellvitgehospital.cat.
• World Neurosurg. 2017 Dec 1; 108: 996.e17-996.e25.

BackgroundComposite ganglioma and pleomorphic xanthoastrocytoma with anaplastic features in both components is an extremely rare glioneuronal tumor. Five cases of anaplastic progression in the glioma component have been reported. These tumors generally affect young patients who have brain tumor-related epilepsy, which are usually located in the temporal lobe or in the cerebellum and may have associated leptomeningeal spreading. Its current optimal treatment consists of maximal safe surgical resection and adjuvant chemoradiotherapy. Overall survival at 5 years is 33% in anaplastic pleomorphic xanthoastrocytoma and 53% in anaplastic ganglioglioma.Case DescriptionWe describe a progression from ganglioglioma to this composite anaplastic entity after 32 months of follow-up, with apparently nontumoral parenchyma separating the 2 components. Polymerase chain reaction showed a wild-type BRAF gene. Seven months after concomitant chemoradiotherapy, radiologic progression led to a second line of chemotherapy, and a third line of chemotherapy was initiated after a subsequent progression at 11 months.ConclusionsThis case may add some evidence in favor of the glioneuronal maldevelopment hypothesis to explain the oncogenesis of these neuroepithelial tumors.Copyright © 2017 Elsevier Inc. All rights reserved.

29 keywords...

hide…