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

Clinical features and surgical outcomes of patients with skull base chordoma: a retrospective analysis of 238 patients
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238例颅底脊索瘤患者临床特征及手术结果的回顾性分析

DOI:
10.3171/2016.9.jns16559
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发表时间:
2017-12-01
影响因子:
4.1
通讯作者:
Zhang, Junting
Zhang, Junting
中科院分区:
医学1区
文献类型:
--
作者:
Wang, Liang;Wu, Zhen;Zhang, Junting

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目的颅底脉络膜炎是一种比较少见的疾病,有关其临床特点的报道较少。此外,与切除范围相关的因素,以及边缘切除对长期生存的价值,对于这种疾病仍然存在疑问。本研究的目的是通过评估其临床特征和手术结果来探讨这些因素。方法回顾性分析238例符合纳入标准的颅底脊索瘤患者。本研究通过统计学描述分析总结了乳腺癌的临床特征、手术入路选择、切除程度和术后并发症,提出了肿瘤部位和骨侵犯的改良分类,通过Pearson χ 2、方差分析、秩和检验和二元Logistic回归分析研究了临床和影像学因素对切除程度的影响,并对手术切除的影响因素进行了分析。采用Kaplan-Meier法评估不同治疗史、肿瘤部位分类、骨浸润程度和肿瘤切除程度患者的总生存率和无进展生存率的差异。结果:本研究包括140名男性和98名女性患者,平均年龄为38.1岁。首发症状以头痛、颈痛(33.2%)和复视(29%)为主。肿瘤部位以蝶斜坡型最多见(59.2%),骨侵犯以内生性脉络膜型最多见(81.5%)。在三分之二的研究人群(78.6%)中进行了外侧开放入路。边缘切除率为66%,其中大体全切除率为11.8%,近全切除率为54.2%。脑膜炎(8%)和脑脊液漏(3.8%)是最常见的并发症。平均随访时间为43.7个月。5年总生存率和无进展生存率分别为76%和45%。肿瘤复发和较大的肿瘤体积(>= 40 cm(3))被确定为边缘切除的危险因素。复发肿瘤和接受病灶内切除的患者有一个更糟糕的长期outcome.CONCLUSIONS肿瘤位置和骨侵犯的分类表现出临床价值。对于体积较小的原发病灶(< 40 cm(3)),更有可能实现边缘切除。由于使用游离脂肪移植物改善了硬脑膜修复,脑脊液漏率下降。原发性脊索瘤患者应行边缘切除或全切除,以获得更好的长期生存率。
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 chi(2), 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(3)) 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(3)). 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.