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患者颅底脊索瘤的临床特征和手术结果:238 例回顾性分析。

Clinical features and surgical outcomes of patients with skull base chordoma: a retrospective analysis of 238 patients.

机构信息

1Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University; and.

2China National Clinical Research Center for Neurological Diseases, Beijing, People's Republic of China.

出版信息

J Neurosurg. 2017 Dec;127(6):1257-1267. doi: 10.3171/2016.9.JNS16559. Epub 2017 Jan 6.

Abstract

OBJECTIVE Skull base chordoma is relatively rare, and a limited number of reports have been published regarding its clinical features. Moreover, the factors associated with extent of resection, as well as the value of marginal resection for long-term survival, are still in question for this disease. The objective of this study was to investigate these factors by evaluating their clinical features and surgical outcomes. METHODS A retrospective analysis of 238 patients with skull base chordomas, who met the inclusion criteria, was performed. This study summarized the clinical features, selection of approaches, degree of resection, and postoperative complications by statistical description analyses; proposed modified classifications of tumor location and bone invasion; studied the contributions of the clinical and radiological factors to the extent of resection by Pearson χ, ANOVA, rank test, and binary logistic regression analysis; and estimated the differences in overall survival and progression-free survival rates with respect to therapeutic history, classification of tumor location, extent of bone invasion, and extent of tumor resection by the Kaplan-Meier method. A p value < 0.05 was considered statistically significant. RESULTS The study included 140 male and 98 female patients with a mean age of 38.1 years. Headache and neck pain (33.2%) and diplopia (29%) were the most common initial symptoms. Sphenoclival type accounted for the largest proportion of tumor location (59.2%); endophytic chordoma was the more common type of bone invasion (81.5%). Lateral open approaches were performed in two-thirds of the study population (78.6%). The rate of marginal resection was 66%, composed of gross-total resection (11.8%) and near-total resection (54.2%). Meningitis (8%) and CSF leakage (3.8%) were the most frequent complications. The mean follow-up period was 43.7 months. The overall survival and progression-free survival rates at 5 years were 76% and 45%, respectively. Recurrent tumor and larger tumor volume (≥ 40 cm) were identified as risk factors of marginal resection. Patients who presented with recurrent tumor and underwent intralesional resection had a worse long-term outcome. CONCLUSIONS The classifications of both tumor location and bone invasion demonstrated clinical value. Marginal resection was more likely to be achieved for primary lesions with smaller volumes (< 40 cm). The rate of CSF leakage declined due to improved dura mater repair with free fat grafts. Marginal resection, or gross-total resection when possible, should be performed in patients with primary chordomas to achieve better long-term survival.

摘要

目的

颅底脊索瘤较为罕见,相关临床特征的报道数量有限。此外,对于该病,扩大切除术的相关影响因素以及切缘阳性对长期生存的价值仍存在争议。本研究旨在通过评估其临床特征和手术结果来探讨这些因素。

方法

对符合纳入标准的 238 例颅底脊索瘤患者进行回顾性分析。本研究通过统计描述分析总结了临床特征、入路选择、切除程度和术后并发症;提出了肿瘤位置和骨侵犯的改良分类;通过 Pearson χ²、方差分析、秩和检验和二项逻辑回归分析研究了临床和影像学因素对切除程度的影响;采用 Kaplan-Meier 法估计治疗史、肿瘤位置分类、骨侵犯程度和肿瘤切除程度对总生存率和无进展生存率的差异。p 值<0.05 被认为具有统计学意义。

结果

研究纳入了 140 例男性和 98 例女性患者,平均年龄为 38.1 岁。头痛和颈部疼痛(33.2%)和复视(29%)是最常见的首发症状。蝶枕型占肿瘤位置的最大比例(59.2%);内生型脊索瘤是更常见的骨侵犯类型(81.5%)。三分之二的患者采用外侧开放入路(78.6%)。切缘阳性率为 66%,包括大体全切除(11.8%)和近全切除(54.2%)。脑膜炎(8%)和脑脊液漏(3.8%)是最常见的并发症。中位随访时间为 43.7 个月。5 年总生存率和无进展生存率分别为 76%和 45%。复发性肿瘤和较大肿瘤体积(≥40cm³)是切缘阳性的危险因素。复发性肿瘤患者行肿瘤内切除术的长期预后更差。

结论

肿瘤位置和骨侵犯的分类具有临床价值。体积较小(<40cm³)的原发性肿瘤更有可能实现切缘阳性。由于使用游离脂肪移植修复硬脑膜,脑脊液漏的发生率有所下降。对于原发性脊索瘤患者,应行切缘阳性或尽可能行大体全切除,以获得更好的长期生存。

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