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. 2023:1405:253-280.
doi: 10.1007/978-3-031-23705-8_9.

Glioneuronal and Neuronal Tumors of the Central Nervous System

Matteo Martinoni  1 , Viscardo Paolo Fabbri  2 , Emanuele La Corte  3 , Mino Zucchelli  4 , Francesco Toni  3   5 , Sofia Asioli  2   6 , Caterina Giannini  7   8
Affiliations

Glioneuronal and Neuronal Tumors of the Central Nervous System

Matteo Martinoni et al. Adv Exp Med Biol. 2023.

Abstract

Glioneuronal and neuronal tumors (GNTs) are rare neoplasms composed of neural and glial elements frequently located in the temporal lobe. Epilepsy is the main symptom and diagnosis mostly occurs before adulthood. The great majority of GNTs are WHO grade I tumors, but anaplastic transformations and forms exist. Their common association with focal cortical dysplasia is well recognized and should be taken into consideration during neurophysiological presurgical and surgical planning since the aim of surgery should be the removal of the tumor and of the entire epileptogenic zone according to anatomo-electrophysiological findings. Surgery still remains the cornerstone of symptomatic GNT, while radiotherapy, chemotherapy, and new target therapies are generally reserved for anaplastic, unresectable, or evolving tumors. Furthermore, since many GNTs show overlapping clinical and neuroradiological features, the definition of specific histopathological, genetic, and molecular characteristics is crucial. Epileptological, oncological, neurosurgical, and pathological issues of these tumors make a multidisciplinary management mandatory.

Keywords: Drug resistant epilepsy; Epilepsy surgery; Glioneuronal tumor; Low grade epilepsy associated tumor; Neuronal tumor.

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