Gaggiotti Claudia, Giammalva Giuseppe Roberto, Raimondi Marco, Florena Ada Maria, Gerardi Rosa Maria, Graziano Francesca, Tumbiolo Silvana, Iacopino Domenico Gerardo, Maugeri Rosario
Department of Biomedicine Neurosciences and Advanced Diagnostics, School of Medicine, University of Palermo, Italy.
Department of Scienze per la Promozione della Salute e Materno Infantile, Pathology Unit, University of Palermo, Italy.
Surg Neurol Int. 2021 Mar 8;12:88. doi: 10.25259/SNI_951_2020. eCollection 2021.
Extraventricular neurocytoma (EVN) is an extremely rare neoplasm of the central nervous system. As reported, it arises in a variety of locations, but mainly within the cerebral hemispheres. Despite its histological similarity with central neurocytoma (CN), EVN occurs outside the ventricular system and, in 2007, was recognized by the World Health Organization as a separate entity.
A 39-year-old man, with a ventriculoperitoneal shunt inserted for communicating hydrocephalus, was admitted at our Unit of Neurosurgery with a 1-month history of gait disturbance, postural instability, speech disorders, and occasional incontinence. Computed tomography scan and magnetic resonance imaging showed a mixed-density neoplasm in the left frontotemporal area, with anterior cerebral falx shift, and perilesional edema. The patient underwent surgical procedure; microsurgical excision of the lesion was performed through left pterional approach. Histopathological and immunohistochemical examination revealed monomorphic round cells of the neuronal lineage, with a percentage of Ki-67 positive nuclei <5% and no evidence of mitosis or necrotic areas. According to radiologic features, this pattern was compatible with the diagnosis of EVN. Patient had a favorable recovery and he is still in follow-up.
Because of their rarity, clinical, radiologic, and histopathological characteristics of EVNs are not yet well defined, as well as the optimal therapeutic management. Whereas EVNs are rarely described in literature, we aimed to share and discuss our experience along with a review of the published literature.
脑室外神经细胞瘤（EVN）是一种极为罕见的中枢神经系统肿瘤。据报道，它可发生于多种部位，但主要位于大脑半球内。尽管其组织学特征与中枢神经细胞瘤（CN）相似，但EVN发生在脑室系统之外，并且在2007年被世界卫生组织确认为一个独立的实体。
一名39岁男性，因交通性脑积水置入了脑室腹腔分流管，因有1个月的步态障碍、姿势不稳、言语障碍及偶尔失禁的病史入住我们的神经外科病房。计算机断层扫描和磁共振成像显示左额颞区有一个混合密度肿瘤，伴有大脑镰前部移位及瘤周水肿。患者接受了手术；通过左翼点入路对病变进行了显微手术切除。组织病理学和免疫组织化学检查显示为神经元谱系的单形性圆形细胞，Ki-67阳性核百分比<5%，且无有丝分裂或坏死区域的证据。根据影像学特征，这种表现符合EVN的诊断。患者恢复良好，仍在随访中。
由于EVN罕见，其临床、影像学和组织病理学特征以及最佳治疗方案尚未明确界定。鉴于文献中对EVN的描述很少，我们旨在分享和讨论我们的经验，并对已发表的文献进行综述。
