Anghileri Elena, Broggi Morgan, Mazzapicchi Elio, Farinotti Mariangela, Botturi Andrea, Tramacere Irene, Marchetti Marcello
Unit of Molecular Neuro-Oncology, Fondazione IRCCS Istituto Neurologico Carlo Besta, 20133 Milan, Italy.
Department of Neurosurgery, Fondazione IRCCS Istituto Neurologico Carlo Besta, 20133 Milan, Italy.
Cancers (Basel). 2022 Mar 2;14(5):1292. doi: 10.3390/cancers14051292.
Gliomas are primary tumors arising from supporting cells of the central nervous system (CNS), usually in the brain. The 2021 World Health Organization (WHO) classifies gliomas as adult-type diffuse gliomas or circumscribed astrocytic gliomas depending on their histology and molecular features. Spinal astrocytic gliomas are very rare, and nowadays no standard of therapy is available. Treatment options are limited: surgery is often not radical, and adjuvant therapies include mostly radiotherapy (RT) or systemic chemotherapy (CHT). There is lack of knowledge about the efficacy and safety of therapies and their multidisciplinary approaches.
A systematic review of the literature from January 2000 to June 2021 was performed, including both clinical trials and observational studies on histological adult primary spinal cord astrocytomas (SCA), with a minimum follow-up of 6 months and reporting the overall survival, progression-free survival or clinical neurological outcome after any therapeutic approach (surgery, RT or CHT). What are the main findings? A total of 1197 citations were identified by the Medline search and additional records; based on our inclusion criteria, 18 studies were included with a total of 285 adult patients. We documented the lack of any clinical trial. What are the conclusions? The available literature data are limited to series/retrospective studies, including heterogeneous patients, i.e., astrocytoma as well as ependymoma or pediatric/adult age, with scanty data on the outcomes of interest. No clinical trials have been run. Due to the rarity of this disease, multicentric clinical trials with molecular investigations are mandatory to better manage such a rare disease.
胶质瘤是起源于中枢神经系统（CNS）支持细胞的原发性肿瘤，通常发生于脑部。2021年世界卫生组织（WHO）根据组织学和分子特征将胶质瘤分类为成人型弥漫性胶质瘤或局限性星形细胞胶质瘤。脊髓星形细胞胶质瘤非常罕见，目前尚无标准治疗方案。治疗选择有限：手术往往不彻底，辅助治疗主要包括放疗（RT）或全身化疗（CHT）。对于治疗的疗效和安全性及其多学科方法缺乏了解。
对2000年1月至2021年6月的文献进行系统综述，包括关于组织学成人原发性脊髓星形细胞瘤（SCA）的临床试验和观察性研究，最短随访时间为6个月，并报告任何治疗方法（手术、RT或CHT）后的总生存期、无进展生存期或临床神经学结果。主要发现有哪些？通过Medline检索和其他记录共识别出1197条引文；根据我们的纳入标准，纳入了18项研究，共有285例成年患者。我们记录了缺乏任何临床试验的情况。结论是什么？现有文献数据仅限于系列研究/回顾性研究，包括异质性患者，即星形细胞瘤以及室管膜瘤或儿童/成人患者，关于感兴趣结局的数据很少。尚未进行临床试验。由于这种疾病罕见，必须开展多中心分子研究的临床试验，以便更好地管理这种罕见疾病。
