Colli B, Al-Mefty O
Division of Neurosurgery, Department of Surgery, Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil.
J Neurosurg. 2001 Dec;95(6):933-43. doi: 10.3171/jns.2001.95.6.0933.
Chordomas are rare tumors that arise from the remnants of the notochord. Because of their deep location, local infiltrative nature, and involvement of surrounding bone, treatment of chordomas is a challenge. In this study the authors analyze the data and prognostic factors obtained during the follow-up period (range 1-150 months, median 38 months) in 53 patients with craniocervical junction chordomas and 10 patients with chondrosarcomas.
Several surgical approaches were used, and some tumor excisions required staged procedures. Survival was calculated according the Kaplan-Meier method. Statistical analysis was performed using Fisher exact, log rank and Kruskal-Wallis tests. Radical/subtotal resection was achieved in 77.8% of the patients. The mortality rate during the follow-up period was 14.3%. In patients harboring chondrosarcoma better 5-year recurrence-free survival (RFS) rates were demonstrated than in those with chordoma (100% and 50.7%. respectively). Histological patterns (typical or chondroid chordomas) and patient age at onset of symptoms had no effect on the RFS rates. Radical/subtotal resections were associated with better RFS rates than partial resection. Adjuvant proton-beam therapy was shown to increase the RFS rates compared with conventional radiotherapy (90.9% and 19.4%, respectively, at 4 years posttreatment). Karyotypically abnormal tumors were associated with the worst RFS rates compared with karyotypically normal lesions (44.5% and 90.3%, respectively, at 3 years). Cases of cranial nerve palsy followed by those with cerebrospinal fluid leakages were the most frequent postoperative complications. Permanent postoperative neurological deficit was observed in 28.6% of the patients.
A better prognosis was observed in patients with chondrosarcoma compared with those harboring chordoma. Histological pattern and patient age at symptom onset were not factors that influenced prognosis in cases of chordoma. Extensive resection and possibly adjuvant proton-beam therapy provided better prognoses for these patients.
脊索瘤是一种起源于脊索残余组织的罕见肿瘤。由于其位置深、具有局部浸润性且累及周围骨骼，脊索瘤的治疗颇具挑战性。在本研究中，作者分析了53例颅颈交界区脊索瘤患者和10例软骨肉瘤患者在随访期（范围1 - 150个月，中位时间38个月）获得的数据及预后因素。
采用了多种手术入路，部分肿瘤切除需要分期进行。根据Kaplan - Meier方法计算生存率。使用Fisher精确检验、对数秩检验和Kruskal - Wallis检验进行统计分析。77.8%的患者实现了根治性/次全切除。随访期内死亡率为14.3%。与脊索瘤患者相比，软骨肉瘤患者的5年无复发生存率（RFS）更高（分别为100%和50.7%）。组织学模式（典型或软骨样脊索瘤）和症状出现时的患者年龄对RFS率无影响。根治性/次全切除与比部分切除更好的RFS率相关。与传统放疗相比，辅助质子束治疗显示可提高RFS率（治疗后4年分别为90.9%和19.4%）。与核型正常的病变相比，核型异常的肿瘤RFS率最差（3年时分别为44.5%和90.3%）。术后最常见的并发症是颅神经麻痹，其次是脑脊液漏。28.6%的患者出现永久性术后神经功能缺损。
与脊索瘤患者相比，软骨肉瘤患者的预后更好。组织学模式和症状出现时的患者年龄不是影响脊索瘤患者预后的因素。广泛切除以及可能的辅助质子束治疗为这些患者提供了更好的预后。
