Tonn Joerg-Christian, Teske Nico, Karschnia Philipp
Department of Neurosurgery, LMU University Hospital, Ludwig-Maximilians-University, Munich, Germany.
German Cancer Consortium (DKTK), Partner Site Munich, Heidelberg, Germany.
Neurooncol Adv. 2024 Feb 13;6(Suppl 3):iii48-iii56. doi: 10.1093/noajnl/vdad166. eCollection 2024 Oct.
Tumors of astrocytic origin represent one of the most frequent entities among the overall rare group of spinal cord gliomas. Initial clinical symptoms are often unspecific, and sensorimotor signs localizing to the spinal cord occur with progressing tumor growth. On MRI, a hyperintense intrinsic spinal cord signal on T-weighted sequences with varying degrees of contrast enhancement raises suspicion for an infiltrative neoplasm. Blood and CSF analysis serves to exclude an infectious process, nutritional deficits, or metabolic disorders. When such other differential diagnoses have been ruled out, a neuropathological tissue-based analysis is warranted to confirm the diagnosis of a spinal cord astrocytoma and guide further patient management. As such, maximal safe resection forms the basis of any treatment. Meticulous preoperative planning is necessary to weigh the potential improvement in survival against the risk of functional deterioration. Intraoperative neuromonitoring and ultrasound may aid in achieving a more extensive resection. Depending on the assigned WHO tumor grade spanning from grade 1 to grade 4, the use of radiotherapy and chemotherapy might be indicated but also wait-and-scan approaches appear reasonable in tumors of lower grade. Close imaging follow-up is necessary given that recurrence inevitably occurs in astrocytomas of grades 2-4. Prognosis is so far dictated by tumor grade and histopathological findings, but also by age and clinical performance of the patient. Targeted therapies resting upon an in-depth tissue analysis are emerging in recurrent tumors, but no prospective study is available so far given the rarity of spinal cord astrocytomas.
星形细胞起源的肿瘤是脊髓胶质瘤这一总体罕见肿瘤组中最常见的类型之一。初始临床症状通常不具特异性,随着肿瘤生长进展会出现定位至脊髓的感觉运动体征。在磁共振成像(MRI)上,T加权序列上脊髓内高强度信号并伴有不同程度的对比增强,提示可能存在浸润性肿瘤。血液和脑脊液分析有助于排除感染性疾病、营养缺乏或代谢紊乱。当排除了其他此类鉴别诊断后,有必要进行基于神经病理组织的分析以确诊脊髓星形细胞瘤并指导进一步的患者管理。因此,最大安全切除是任何治疗的基础。细致的术前规划对于权衡生存获益与功能恶化风险至关重要。术中神经监测和超声可能有助于实现更广泛的切除。根据世界卫生组织(WHO)指定的肿瘤分级,范围从1级到4级,可能需要使用放疗和化疗,但对于低级别肿瘤,等待观察扫描的方法似乎也是合理的。鉴于2 - 4级星形细胞瘤不可避免地会复发,密切的影像学随访是必要的。目前,预后取决于肿瘤分级和组织病理学结果,也取决于患者的年龄和临床表现。基于深入组织分析的靶向治疗正在复发性肿瘤中出现,但鉴于脊髓星形细胞瘤的罕见性,目前尚无前瞻性研究。