Neyazi Belal, Haghikia Aiden, Mawrin Christian, Hattingen Elke, Vordermark Dirk, Sandalcioglu I Erol
University Clinic for Neurosurgery, Otto-von-Guericke Universität, Magdeburg; University Department of Neurology, Otto-von-Guericke Universität, Magdeburg; Department of Neuropathology, Otto-von-Guericke Universität, Magdeburg; Department of Neuroradiology, University Hospital Frankfurt, Frankfurt am Main; University Clinic for Radiation Therapy, Otto-von-Guericke Universität, Magdeburg; Department of Radiotherapy, Faculty of Medicine, Martin Luther University Halle-Wittenberg, Halle (Saale).
Dtsch Arztebl Int. 2024 Dec 13;121(25):840-846. doi: 10.3238/arztebl.m2024.0213.
Intramedullary tumors are a subgroup of spinal tumors and are associated with high morbidity and mortality. The estimated incidence of spinal tumors in general is 0.74 to 1.6 per 100 000 persons per year, with intramedullary tumors making up 10% to 30% of the total. The diagnosis is often delayed because of the insidious onset of symptoms, which are often nonspecific at first.
This review is based on pertinent publications about intramedullary tumors that were retrieved by a selective search in the PubMed database.
Intramedullary tumors often cause diffuse neurologic symptoms of gradually increasing severity, progressing, in advanced cases, to a complete spinal cord transection syndrome. Magnetic resonance imaging of the spine without and with intravenous contrast is the standard diagnostic technique. The histopathological origin of most intramedullary tumors is from glial cells, but other types of intramedullary tumor exist as well. The primary treatment of all intramedullary tumors is surgical resection. 9% to 34% of patients may experience a worse neurological deficit after surgery than before, but such problems resolve completely in 25% to 41% of cases. The extent of resection is the main factor affecting the risk of tumor recurrence and progression. The extent of resection also determines the possible indication for adjuvant treatment, which is needed, in particular, for high-grade and subtotally resected tumors, and for those that display progression. The treatment of intramedullary tumors is based on case series, retrospective analyses, and case reports, as randomized trials are lacking.
Patients with intramedullary tumors should be cared for, as much as possible, in the setting of prospective, uniform studies of their spontaneous course and the outcomes after treatment. This will yield better evidence on the treatment of these tumors in the future.
髓内肿瘤是脊柱肿瘤的一个亚组,与高发病率和死亡率相关。一般来说,脊柱肿瘤的估计发病率为每年每10万人中有0.74至1.6例,髓内肿瘤占总数的10%至30%。由于症状隐匿起病,且起初往往不具有特异性,诊断常常延迟。
本综述基于通过在PubMed数据库中进行选择性检索获得的有关髓内肿瘤的相关出版物。
髓内肿瘤常引起严重程度逐渐增加的弥漫性神经症状,在晚期病例中进展为完全性脊髓横断综合征。脊柱的磁共振成像(有无静脉造影剂)是标准的诊断技术。大多数髓内肿瘤的组织病理学起源是神经胶质细胞,但也存在其他类型的髓内肿瘤。所有髓内肿瘤的主要治疗方法是手术切除。9%至34%的患者术后神经功能缺损可能比术前更严重,但在25%至41%的病例中此类问题可完全解决。切除范围是影响肿瘤复发和进展风险的主要因素。切除范围还决定了辅助治疗的可能指征,特别是对于高级别和次全切除的肿瘤以及那些出现进展的肿瘤。由于缺乏随机试验,髓内肿瘤的治疗基于病例系列、回顾性分析和病例报告。
髓内肿瘤患者应尽可能在前瞻性、统一的研究背景下接受治疗,研究其自然病程和治疗后的结果。这将为未来这些肿瘤的治疗提供更好的证据。