TY - JOUR
T1 - Glioneuronal and Neuronal Tumors of the Central Nervous System
AU - Martinoni, Matteo
AU - Fabbri, Viscardo Paolo
AU - La Corte, Emanuele
AU - Zucchelli, Mino
AU - Toni, Francesco
AU - Asioli, Sofia
AU - Giannini, Caterina
N1 - Publisher Copyright:
© 2023. Springer Nature Switzerland AG.
PY - 2023
Y1 - 2023
N2 - 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.
AB - 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.
KW - Drug resistant epilepsy
KW - Epilepsy surgery
KW - Glioneuronal tumor
KW - Low grade epilepsy associated tumor
KW - Neuronal tumor
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U2 - 10.1007/978-3-031-23705-8_9
DO - 10.1007/978-3-031-23705-8_9
M3 - Article
C2 - 37452941
AN - SCOPUS:85164756370
SN - 0065-2598
VL - 1405
SP - 253
EP - 280
JO - Advances in experimental medicine and biology
JF - Advances in experimental medicine and biology
ER -