Background and Objectives: Primary central nervous system (CNS) tumors are often associated with relatively poor outcomes. Data on the epidemiology and outcome of CNS tumors in Jordan are scarce. We aim to report the epidemiology and outcome of primary CNS tumors of patients managed at a comprehensive cancer care center in Jordan. Methods: We performed a retrospective chart review of all Jordanian patients with a primary CNS tumor diagnosis who were managed at the center between July 2003 and June 2019. We included all entities described in the 2021 CNS WHO classification system, in addition to pituitary neuroendocrine tumors (PitNETs). We used the Kaplan–Meier method to estimate the 1-year, 2-year, and 5-year overall survival (OS) rates for each entity. Results and Findings: We included 2094 cases. The numbers of pediatrics and adults were 652 (31.1%) and 1442 (68.9%), respectively. The three most common groups of tumors were “gliomas, glioneuronal tumors, and neuronal tumors” (n= 1200 [57.30%]), followed by meningiomas (n= 261 [12.5%]), embryonal tumors (n= 234 [11.2%]). The three most common tumor families were adult-type diffuse gliomas (n= 709 [33.8%]), medulloblastoma (n= 199 [9.5%]), and circumscribed astrocytic gliomas (n= 183 [8.7%]). The median survival for the entire cohort was 97 months (95CI; 81–112). Survival was significantly worse for males and adults compared to their respective counterparts. Among the most common tumor group, “gliomas, glioneuronal tumors, and neuronal tumors”, OS rates for adult-type diffuse gliomas were significantly lower than all other types. Overall, adult gliomas with IDH-mutations had a survival advantage over wildtype cases (IDH-mutant 1-year OS, 89% [82–97%] vs. IDH-wildtype 1-year OS, 60% [52–70%];p< 0.001). Conclusions: We present a detailed analysis of the primary CNS tumors diagnosed in the largest cancer center in Jordan between 2003 and 2019. We compared the epidemiology and overall survival of these patients to worldwide estimates and found the epidemiology and outcome of these tumors comparable to worldwide trends.
背景与目的:原发性中枢神经系统(CNS)肿瘤通常预后较差。约旦地区关于CNS肿瘤流行病学及预后的数据较为缺乏。本研究旨在报告约旦一家综合性癌症治疗中心收治的原发性CNS肿瘤患者的流行病学特征及临床结局。方法:我们对2003年7月至2019年6月期间在该中心诊治的所有约旦籍原发性CNS肿瘤患者进行了回顾性病历分析。纳入标准涵盖2021年CNS世界卫生组织分类系统中描述的所有肿瘤类型,并包括垂体神经内分泌肿瘤(PitNETs)。采用Kaplan-Meier法估算各类肿瘤的1年、2年及5年总生存率。结果:共纳入2094例病例,其中儿童652例(31.1%),成人1442例(68.9%)。最常见的三大肿瘤类别为"胶质瘤、胶质神经元肿瘤与神经元肿瘤"(1200例,57.30%),其次为脑膜瘤(261例,12.5%)和胚胎性肿瘤(234例,11.2%)。最常见的三种肿瘤亚型分别为成人型弥漫性胶质瘤(709例,33.8%)、髓母细胞瘤(199例,9.5%)和局限性星形细胞胶质瘤(183例,8.7%)。全队列中位生存期为97个月(95%CI:81-112)。男性与成人患者的生存预后显著差于女性和儿童患者。在"胶质瘤、胶质神经元肿瘤与神经元肿瘤"这一主要类别中,成人型弥漫性胶质瘤的总生存率显著低于其他所有亚型。总体而言,IDH突变型成人胶质瘤较野生型患者具有生存优势(IDH突变型1年总生存率89%[82-97%] vs 野生型60%[52-70%],p<0.001)。结论:本研究对2003年至2019年间约旦最大癌症中心诊断的原发性CNS肿瘤进行了详细分析。通过将患者流行病学特征与总生存数据与国际标准比较,发现该地区肿瘤流行病学模式与临床结局与全球趋势基本一致。