Objective:Central neurocytomas (CNs), classified as CNS (central nervous system) grade 2 tumors, are exceptionally rare tumors, accounting for approximately 0.1–0.5% of all intracranial neoplasms, and are typically characterized by a benign clinical course and frequent association with hydrocephalus. This study aims to present a comprehensive analysis of surgical and adjuvant therapies for CN.Methods:The study comprised all patients who underwent microsurgical tumor removal in our center over the past decade (2013–2023). Clinical manifestations, surgical and adjuvant therapy approaches, MRI and histological findings, clinical outcomes, and recurrence-free survival were evaluated.Results:A total of eleven patients (six men, mean age of 28.0 years; five women, mean age of 53.6 years) underwent surgical treatment. Intraventricular tumors were the most common (72.7%, n = 8). The predominant presenting symptoms were headache and visual disturbances. All tumors exhibited contrast enhancement on MRI. Hydrocephalus was present in five patients. The Ki67 proliferation index ranged from 2% to 10%, with nine patients exhibiting Ki67 > 3%. The median recurrence-free survival was 38.0 months (IQR: 25.0–53.0). The most severe postoperative complications included aphasia, hemiparesis, and memory impairment, resulting in a postoperative Karnofsky Performance Status (KPS) below 70% in five patients. Follow-up assessments showed significant symptomatic improvement in all affected patients.Conclusions:Gross total resection is the recommended first-line therapy with favorable neurological outcomes and for atypical CN as well. Adjuvant radiotherapy should be reserved for tumor progression and recurrence. The role of adjuvant chemotherapy remains unclear, but it may be an option for CN with a high proliferation index.
目的:中枢神经细胞瘤(CNs)被归类为中枢神经系统2级肿瘤,是一种极为罕见的肿瘤,约占所有颅内肿瘤的0.1–0.5%,通常具有良性临床病程,且常伴有脑积水。本研究旨在对中枢神经细胞瘤的手术及辅助治疗进行全面分析。 方法:本研究纳入了过去十年(2013–2023年)在我中心接受显微外科肿瘤切除术的所有患者。对临床表现、手术及辅助治疗方法、MRI和组织学结果、临床结局以及无复发生存期进行了评估。 结果:共有11名患者(6名男性,平均年龄28.0岁;5名女性,平均年龄53.6岁)接受了手术治疗。脑室内肿瘤最为常见(72.7%,n = 8)。主要症状为头痛和视觉障碍。所有肿瘤在MRI上均显示强化。5名患者存在脑积水。Ki67增殖指数范围为2%至10%,其中9名患者的Ki67 > 3%。中位无复发生存期为38.0个月(IQR:25.0–53.0)。最严重的术后并发症包括失语、偏瘫和记忆障碍,导致5名患者术后卡氏功能状态评分(KPS)低于70%。随访评估显示所有受影响患者的症状均有显著改善。 结论:大体全切除是推荐的一线治疗方法,对于非典型中枢神经细胞瘤也能带来良好的神经功能预后。辅助放疗应保留用于肿瘤进展和复发的情况。辅助化疗的作用尚不明确,但对于高增殖指数的中枢神经细胞瘤可能是一种选择。