Intramedullary primary spinal cord tumors are rare in adults and their classification has recently evolved. Their treatment most frequently relies on maximal safe surgical resection. Herein, we review, in light of the WHO 2021 classification of central nervous system tumors, the knowledge regarding the role of radiotherapy and systemic treatments in spinal ependymomas, spinal astrocytomas (pilocytic astrocytoma, diffuse astrocytoma, spinal glioblastoma IDH wildtype, diffuse midline glioma H3-K27M altered, and high-grade astrocytoma with piloid features), neuro-glial tumors (ganglioglioma and diffuse leptomeningeal glioneuronal tumor), and hemangioblastomas. In spinal ependymomas, radiotherapy is recommended for incompletely resected grade 2 tumors, grade 3 tumors, and recurrent tumors not amenable to re-surgery. Chemotherapy is used in recurrent cases. In spinal astrocytomas, radiotherapy is recommended for incompletely resected grade 2 astrocytomas and grade 3 or 4 tumors as well as recurrent tumors. Chemotherapy is indicated for newly diagnosed high-grade astrocytomas and recurrent cases. In hemangioblastomas not amenable to surgery, radiotherapy is an effective alternative option. Targeted therapies are playing an increasingly important role in the management of some intramedullary primary spinal cord tumor subtypes. BRAF and/or MEK inhibitors have demonstrated efficacy in pilocytic astrocytomas and glioneuronal tumors, belzutifan in von Hippel–Lindau-related hemangioblastomas, and promising results have been reported with ONC201 in diffuse midline glioma H3-K27M altered.
髓内原发性脊髓肿瘤在成人中较为罕见，其分类标准近年来不断更新。此类肿瘤的治疗主要依赖于最大范围的安全手术切除。本文基于世界卫生组织2021年中枢神经系统肿瘤分类标准，系统综述了放射治疗及全身性治疗在脊髓室管膜瘤、脊髓星形细胞瘤（毛细胞型星形细胞瘤、弥漫性星形细胞瘤、IDH野生型脊髓胶质母细胞瘤、H3-K27M突变型弥漫性中线胶质瘤、伴毛样特征的高级别星形细胞瘤）、神经胶质混合性肿瘤（节细胞胶质瘤与弥漫性软脑膜胶质神经元肿瘤）以及血管母细胞瘤治疗中的作用。对于脊髓室管膜瘤，放射治疗推荐用于未完全切除的2级肿瘤、3级肿瘤以及无法再次手术的复发性肿瘤。化学治疗适用于复发病例。在脊髓星形细胞瘤中，放射治疗适用于未完全切除的2级星形细胞瘤、3级或4级肿瘤以及复发性肿瘤。化学治疗适用于新诊断的高级别星形细胞瘤和复发病例。对于无法手术的血管母细胞瘤，放射治疗是有效的替代选择。靶向治疗在某些髓内原发性脊髓肿瘤亚型的治疗中正发挥日益重要的作用：BRAF和/或MEK抑制剂在毛细胞型星形细胞瘤和胶质神经元肿瘤中显示出疗效，belzutifan用于治疗von Hippel-Lindau病相关血管母细胞瘤，而ONC201在H3-K27M突变型弥漫性中线胶质瘤中已报道了具有前景的治疗效果。
肿瘤(癌症)患者之家