Neurosurg Focus. 2024 May;56(5):E6. doi: 10.3171/2024.2.FOCUS23710.
Chordomas are a rare and relatively slow-growing malignancy of notochordal origin with a nearly 50% recurrence rate. Chordomas of the cervical spine are particularly challenging tumors given surrounding vital anatomical structures. Although standard in other areas of the spine, en bloc resection of cervical chordomas is exceedingly difficult and carries the risk of significant postoperative morbidity. Here, the authors present their institutional experience with 13 patients treated with a structure-sparing radical resection and adjuvant radiation for cervical chordomas.
Records of the standing senior author and institutional database of spinal surgeries were retrospectively reviewed for surgically managed cervical and high thoracic chordomas between 1997 and 2022. Chordomas whose epicenter was cervical but touched the clivus or had extension to the thoracic spine were included in this series. Clinical and operative data were gathered and analyzed for the index surgery and any revisions needed. Outcome metrics such as recurrence rates, complication rates, functional status, progression-free interval (PFI) and overall survival (OS) were evaluated.
The median patient age at diagnosis was 57 (range 32-80) years. The median modified Rankin Scale (mRS) score at the time of presentation was 1 (range 0-4). Approximately 40% of tumors were located in the upper cervical spine (occiput-C2). The median time from diagnosis to surgery was 74.5 (range 10-483) days. Gross-total resection was achieved in just under 40% of patients. All patients received adjuvant radiotherapy. The mean duration of follow-up was 4.09 years, with a mean PFI of 3.80 (range 1.16-13.1) years. Five patients experienced recurrence (38.5%). The mean OS was 3.44 years. Three patients died during the follow-up period; 2 due to disease progression and 1 died in the immediate postoperative period. One patient was lost to follow-up. A significant positive relationship was identified between high cervical tumor location and disease recurrence (p = 0.021).
While en bloc resection is appropriate and feasible for tumors in the sacral spine, the cervical region poses a significant technical challenge and is associated with increased postoperative morbidity. Radical resection may allow for achievement of negative operative margins and, along with sparing postoperative morbidity following resection of cervical chordomas, maintaining a similar rate of recurrence when compared with en bloc resection while preserving quality of life.
脊索瘤是一种罕见且生长缓慢的源自脊索的恶性肿瘤，复发率接近 50%。由于周围存在重要的解剖结构，颈椎脊索瘤是极具挑战性的肿瘤。尽管在脊柱的其他区域标准治疗为整块切除术，但颈椎脊索瘤的整块切除术极其困难，并且有发生重大术后并发症的风险。在这里，作者介绍了他们机构对 13 例采用保留结构的根治性切除术和辅助放疗治疗颈椎脊索瘤患者的经验。
对 1997 年至 2022 年间接受手术治疗的颈椎和胸高位脊索瘤的资深作者的病例记录和机构脊柱手术数据库进行回顾性分析。本研究纳入了肿瘤中心点位于颈椎但触及斜坡或延伸至胸椎的脊索瘤。收集并分析索引手术和任何修订的临床和手术数据。评估复发率、并发症发生率、功能状态、无进展间隔（PFI）和总生存率（OS）等预后指标。
诊断时患者的中位年龄为 57 岁（范围 32-80 岁）。就诊时中位改良 Rankin 量表（mRS）评分为 1 分（范围 0-4 分）。约 40%的肿瘤位于颈椎上部（枕骨-C2）。从诊断到手术的中位时间为 74.5 天（范围 10-483 天）。接近 40%的患者达到了大体全切除。所有患者均接受了辅助放疗。中位随访时间为 4.09 年，平均 PFI 为 3.80 年（范围 1.16-13.1 年）。5 例患者复发（38.5%）。平均 OS 为 3.44 年。3 例患者在随访期间死亡；2 例死于疾病进展，1 例死于术后即刻。1 例患者失访。高颈椎肿瘤位置与疾病复发之间存在显著的正相关关系（p=0.021）。
尽管整块切除术适用于骶骨脊柱的肿瘤，但颈椎区域具有显著的技术挑战性，并且与术后发病率增加相关。根治性切除术可能实现阴性手术切缘，并在保留术后发病率的情况下保留颈椎脊索瘤的生活质量，与整块切除术相比，维持相似的复发率。
