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Published in: Neurological Sciences 8/2020

01-08-2020 | Glioma | Original Article

Gliomatosis cerebri (GC) or GC-like? A picture to be reconsidered in neuro-oncology based on large retrospective analysis of GC series

Authors: Elena Anghileri, Carla Schettino, Bianca Pollo, Mariangela Farinotti, Antonio Silvani, Rosina Paterra, Monica Patanè, Francesco DiMeco, Maria Grazia Bruzzone, Marica Eoli, Valeria Cuccarini

Published in: Neurological Sciences | Issue 8/2020

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Abstract

Introduction

Gliomatosis cerebri (GC), defined until 2016 as a distinct astrocytic glioma entity, has been removed from the 2016 World Health Organization classification of tumors of the central nervous system. However, its identity is still debated.

Materials and methods

We retrospectively present 122 patients, including a subgroup with histology confirmation (n = 75, cohort b).

Results

Radiological features showed extension limited to 3 lobes in 31%; bilateral, midline, and basal ganglia and subtentorial involvement in 95%, 52%, 84%, and 60%, respectively; and contrast enhancement in 59.5%. Perioperative mortality occurred in 4%. Histology concluded for grades II, III, and IV, respectively, in 31%, 35%, and 22% (not specified in 12%). Thirty-one percent had isocitrate dehydrogenase (IDH) 1 mutation. Treatments included radiotherapy in 51.2% and chemotherapy in 74.5%. Median overall survival was 17 months. Negative prognostic factors for survival were older age, poorer Karnofsky Performance Scale (KPS), subtentorial, midline and disseminated disease, and lack of chemotherapy, at univariate analysis. At multivariate analysis, KPS ≥ 80, chemotherapy, and subtentorial and disseminated disease remained prognostic (p < 0.0001). For cohort b, same prognostic factors were confirmed, except for midline location, at univariate analysis; at multivariate analysis, only KPS ≥ 80 and chemotherapy remained prognostic (p < 0.0001).

Conclusion

We described clinical, neuroimaging, management, and histomolecular features of one of the largest GC series. We identified KPS ≥ 80, radiological pattern as subtentorial localization and dissemination, and chemotherapy as prognostic factors, at multivariate analysis. Planning prospective study, associated to focused genetic assays, could help to clarify if GC has specific features that may result in the identification as a separate entity from other gliomas.
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Metadata
Title
Gliomatosis cerebri (GC) or GC-like? A picture to be reconsidered in neuro-oncology based on large retrospective analysis of GC series
Authors
Elena Anghileri
Carla Schettino
Bianca Pollo
Mariangela Farinotti
Antonio Silvani
Rosina Paterra
Monica Patanè
Francesco DiMeco
Maria Grazia Bruzzone
Marica Eoli
Valeria Cuccarini
Publication date
01-08-2020
Publisher
Springer International Publishing
Published in
Neurological Sciences / Issue 8/2020
Print ISSN: 1590-1874
Electronic ISSN: 1590-3478
DOI
https://doi.org/10.1007/s10072-020-04288-7

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