Departments of1Neurosurgery.
2Radiation Oncology, and.
Neurosurg Focus. 2024 May;56(5):E14. doi: 10.3171/2024.2.FOCUS2419.
Chordomas are locally aggressive neoplasms of the spine or skull base that arise from embryonic remnants of the notochord. Intradural chordomas represent a rare subset of these neoplasms, and few studies have described intradural chordomas in the spine. This review evaluates the presentation, management, and outcomes of intradural spinal chordomas.
A systematic review of PubMed/MEDLINE, EMBASE, Cochrane Library, Scopus, and Web of Science was performed. Studies describing at least 1 case of intradural chordomas anywhere in the spine were included. Extracted details included presenting symptoms, radiological findings, treatment course, follow-up, and disease progression.
Thirty-one studies, with a total of 41 patients, were included in this review. Seventy-six percent (31/41) of patients had primary intradural tumors, whereas 24% (10/41) presented with metastasis. The most common signs and symptoms were pain (n = 27, 66%); motor deficits (n = 20, 49%); sensory deficits (n = 17, 42%); and gait disturbance (n = 10, 24%). The most common treatment for intradural chordoma was resection and postoperative radiotherapy. Sixty-six percent (19/29) of patients reported improvement or complete resolution of symptoms after surgery. The recurrence rate was 37% (10/27), and the complication rate was 25% (6/24). The median progression-free survival was 24 months (range 4-72 months). Four patient deaths were reported. The median follow-up time was 12 months (range 13 days-84 months).
Treatment of intradural spinal chordomas primarily involves resection and radiotherapy. A significant challenge and complication in management is spinal tumor seeding after resection, with 9 studies proposing seeding as a mechanism of tumor metastasis in 11 cases. Factors such as tumor size, Ki-67 positivity, and distant metastasis may correlate with worse outcomes and demonstrate potential as prognostic indicators for intradural spinal chordomas. Further research is needed to improve understanding of this tumor and develop optimal treatment paradigms for these patients.
脊索瘤是一种源自脊索胚胎残余物的脊柱或颅底局部侵袭性肿瘤。硬脊膜内脊索瘤是这些肿瘤的一个罕见亚型，很少有研究描述过脊柱内的硬脊膜内脊索瘤。本综述评估了硬脊膜内脊髓脊索瘤的表现、治疗和结果。
对 PubMed/MEDLINE、EMBASE、Cochrane 图书馆、Scopus 和 Web of Science 进行了系统的文献回顾。纳入至少描述 1 例脊柱任何部位硬脊膜内脊索瘤的病例报告。提取的详细信息包括首发症状、影像学表现、治疗过程、随访和疾病进展。
本综述共纳入 31 项研究，总计 41 例患者。76%（31/41）的患者为原发性硬脊膜内肿瘤，24%（10/41）为转移瘤。最常见的症状是疼痛（n=27，66%）、运动功能障碍（n=20，49%）、感觉障碍（n=17，42%）和步态障碍（n=10，24%）。硬脊膜内脊索瘤的最常见治疗方法是手术切除和术后放疗。66%（19/29）的患者术后症状改善或完全缓解。复发率为 37%（10/27），并发症发生率为 25%（6/24）。无进展生存期的中位数为 24 个月（范围 4-72 个月）。有 4 例患者死亡。中位随访时间为 12 个月（范围 13 天-84 个月）。
硬脊膜内脊髓脊索瘤的治疗主要包括手术切除和放疗。切除后脊柱肿瘤种植是管理中的一个重大挑战和并发症，9 项研究在 11 例中提出种植是肿瘤转移的机制。肿瘤大小、Ki-67 阳性率和远处转移等因素可能与预后不良相关，并可能作为硬脊膜内脊髓脊索瘤的预后指标。需要进一步研究以提高对这种肿瘤的认识，并为这些患者制定最佳的治疗方案。
