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Neuronal and mixed neuronal–glial tumours 

Neuronal and mixed neuronal–glial tumours
Chapter:
Neuronal and mixed neuronal–glial tumours
Author(s):

Riccardo Soffietti

, Hugues Duffau

, Glenn Bauman

, and David Walker

DOI:
10.1093/med/9780199651870.003.0008
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date: 27 May 2020

Neuronal and mixed neuronal-glial tumours are rare tumours of the central nervous system that occur more commonly in children. Despite a generally benign course, most tumours cause medically intractable seizures, and have been denominated as ‘long-term epilepsy-associated tumours’. The World Health Organization classification distinguishes nine histological variants: dysplastic gangliocytoma of the cerebellum/Lhermitte–Duclos disease, desmoplastic infantile astrocytoma and ganglioglioma, dysembryoplastic neuroepithelial tumour, gangliocytoma and ganglioglioma, central neurocytoma and extraventricular neurocytoma, cerebellar liponeurocytoma, papillary glioneuronal tumour, rosette-forming glioneuronal tumour of the fourth ventricle, and spinal paraganglioma. Early surgery with complete resection may significantly improve the likelihood of postoperative epilepsy freedom. Conformal radiotherapy can be considered in case of patients with incompletely resected symptomatic tumours, atypical or high-grade tumours, or in the case of multiple recurrences despite resections. The role of chemotherapy in these lesions remains poorly defined, while targeted therapies are now available to impact some molecular alterations.

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