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Natural course and prognosis of anaplastic gangliogliomas: a multicenter retrospective study of 43 cases from the French Brain Tumor Database

Louis-Marie Terrier Luc Bauchet 1 Valérie Rigau 2 Aymeric Amelot 3 Sonia Zouaoui 4 Isabelle Filipiak 5 Agnès Caille 6 Fabien Almairac 7 Marie-Hélène Aubriot-Lorton 8 Anne-Marie Bergemer-Fouquet Eric Bord Philippe Cornu Alain Czorny Phong Dam Hieu 9, 10 Bertrand Debono 11 Marie-Bernadette Delisle 12 Evelyne Emery 13 Walid Farah 14 Guillaume Gauchotte 15 Catherine Godfraind 16 Jacques Guyotat 17 Bernard Irthum 18 Kevin Janot Pierre-Jean Le Reste 19 Dominique Liguoro Hugues Loiseau 20 Guillaume Lot Vincent Lubrano 21 Emmanuel Mandonnet 22 Philippe Menei 23 Philippe Metellus 24 Serge Milin 25 Bertrand Muckenstrum Hugues Roche Audrey Rousseau 26 Emmanuel Uro-Coste Anne Vital 27 Jimmy Voirin Michel Wager 28 Marc Zanello 29, 30 Patrick François Stéphane Velut 31 Pascale Varlet 29, 30 Dominique Figarella-Branger 32 Johan Pallud 29, 30 Ilyess Zemmoura 31, * 
* Corresponding author
26 CRCINA-ÉQUIPE 17 - Design and Application of Innovative Local Treatments in Glioblastoma
CRCINA - Centre de Recherche en Cancérologie et Immunologie Nantes-Angers
Abstract : Background - Anaplastic gangliogliomas (GGGs) are rare tumors whose natural history is poorly documented. We aimed to define their clinical and imaging features and to identify prognostic factors. Methods - Consecutive cases of anaplastic GGGs in adults prospectively entered into the French Brain Tumor Database between March 2004 and April 2014 were screened. After diagnosis was confirmed by pathological review, clinical, imaging, therapeutic, and outcome data were collected retrospectively. Results - Forty-three patients with anaplastic GGG (median age, 49.4 y) from 18 centers were included. Presenting symptoms were neurological deficit (37.2%), epileptic seizure (37.2%), or increased intracranial pressure (25.6%). Typical imaging findings were unifocal location (94.7%), contrast enhancement (88.1%), central necrosis (43.2%), and mass effect (47.6%). Therapeutic strategy included surgical resection (95.3%), adjuvant radiochemotherapy (48.8%), or radiotherapy alone (27.9%). Median progression-free survival (PFS) and overall survival (OS) were 8.0 and 24.7 months, respectively. Three- and 5-year tumor recurrence rates were 69% and 100%, respectively. The 5-year survival rate was 24.9%. Considering unadjusted significant prognostic factors, tumor midline crossing and frontal location were associated with shorter OS. Temporal and parietal locations were associated with longer and shorter PFS, respectively. None of these factors remained statistically significant in multivariate analysis. Conclusions - We report a large series providing clinical, imaging, therapeutic, and prognostic features of adult patients treated for an intracerebral anaplastic GGG. Our results show that pathological diagnosis is difficult, that survivals are only slightly better than for glioblastomas, and that complete surgical resection followed with adjuvant chemoradiotherapy offers longer survival.
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Submitted on : Wednesday, February 27, 2019 - 3:42:06 PM
Last modification on : Wednesday, November 23, 2022 - 6:10:04 PM

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Louis-Marie Terrier, Luc Bauchet, Valérie Rigau, Aymeric Amelot, Sonia Zouaoui, et al.. Natural course and prognosis of anaplastic gangliogliomas: a multicenter retrospective study of 43 cases from the French Brain Tumor Database. Neuro-Oncology, 2017, 19 (5), pp.678-688. ⟨10.1093/neuonc/now186⟩. ⟨hal-02051153⟩



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