. Lee Moffitt Cancer Center, Department of Neuro-Oncology, Tampa, FL 33612, USA.
J Natl Compr Canc Netw. 2011 Apr;9(4):434-47. doi: 10.6004/jnccn.2011.0039.
Primary spinal cord tumors represent 4.5% of all central nervous system neoplasms. They are either intradural intramedullary or intradural extramedullary. Intramedullary tumors are predominantly intrinsic gliomas (astrocytomas and ependymomas). Spinal ependymomas can usually be completely removed by separating the tumor from the spinal cord and, when complete, no further therapy is required. Astrocytomas, by contrast, infiltrate the myelon, and therefore surgery is frequently incomplete. Intradural extramedullary tumors are mostly benign (WHO grade 1) and comprise either peripheral nerve sheath tumors (neurofibromas and schwannomas) or meningiomas. Complete resection can be performed on both lesions and is often curative. Radiotherapy is indicated for primary malignant tumors (WHO grade 3 and higher) and for patients in whom surgery is contraindicated. For grade 1 and 2 tumors, the role of radiotherapy is controversial. Chemotherapy is reserved for recurrent primary spinal cord tumors with no other options. However, the lack of clinical trials for these tumors is problematic. Consequently, treatment is similar to that for intracranial histologies. Early recognition of the signs and symptoms of primary spinal cord tumors facilitates early treatment, potentially minimizes neurologic morbidity, and improves outcome. Primary treatment for almost all spinal cord tumors is surgery, with predictors of outcome being preoperative functional status, grade of tumor, and extent of resection.
原发性脊髓肿瘤占所有中枢神经系统肿瘤的 4.5%。它们要么是硬脊膜内髓内肿瘤,要么是硬脊膜外髓外肿瘤。髓内肿瘤主要是内在的神经胶质瘤(星形细胞瘤和室管膜瘤)。脊髓室管膜瘤通常可以通过将肿瘤与脊髓分离来完全切除,如果完全切除,则无需进一步治疗。相比之下,星形细胞瘤浸润脊髓,因此手术往往不彻底。硬脊膜外髓外肿瘤大多为良性(WHO 1 级),包括周围神经鞘肿瘤(神经纤维瘤和神经鞘瘤)或脑膜瘤。这两种病变都可以进行完全切除,通常是治愈性的。放疗适用于原发性恶性肿瘤(WHO 3 级及以上)和手术禁忌的患者。对于 1 级和 2 级肿瘤,放疗的作用存在争议。对于没有其他选择的复发性原发性脊髓肿瘤,保留化疗。然而,这些肿瘤缺乏临床试验是有问题的。因此,治疗方法类似于颅内组织学。早期识别原发性脊髓肿瘤的体征和症状有助于早期治疗,最大限度地减少神经功能障碍,并改善预后。几乎所有脊髓肿瘤的主要治疗方法都是手术,其预后预测因素包括术前功能状态、肿瘤分级和切除范围。