Division of Neurosurgery, IRCCS Istituto delle Scienze Neurologiche di Bologna, Bologna, Italy.
Surgical Pathology Section, IRCCS Istituto delle Scienze Neurologiche di Bologna, Bologna, Italy.
Adv Exp Med Biol. 2023;1405:253-280. doi: 10.1007/978-3-031-23705-8_9.
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.
胶质神经元肿瘤（GNTs）是一种罕见的肿瘤，由神经和神经胶质成分组成，通常位于颞叶。癫痫是主要症状，大多数诊断发生在成年前。GNTs 绝大多数为 WHO 分级 I 肿瘤，但也存在间变形式。其与局灶性皮质发育不良的常见关联已得到充分认识，应在神经生理术前和手术规划中考虑到这一点，因为手术的目的应根据解剖-电生理发现切除肿瘤和整个致痫区。手术仍然是治疗症状性 GNT 的基石，而放疗、化疗和新的靶向治疗通常保留给间变、不可切除或进展性肿瘤。此外，由于许多 GNTs 表现出重叠的临床和神经影像学特征，因此确定特定的组织病理学、遗传学和分子特征至关重要。这些肿瘤的癫痫学、肿瘤学、神经外科学和病理学问题使得多学科管理成为必要。
