1Neurosurgical Research Network (NRN), Universal Scientific Education and Research Network (USERN), Tehran University of Medical Sciences, Tehran, Iran.
2Department of Neurosurgery, Tehran University of Medical Sciences, Tehran, Iran.
Neurosurg Focus. 2024 May;56(5):E10. doi: 10.3171/2024.2.FOCUS249.
Chordoma is a primary bone tumor with limited literature on its management because of its rarity. Resection, while considered the first-line treatment, does not always provide adequate tumor control. In this systematic review, the authors aimed to provide comprehensive insights by managing these tumors with stereotactic radiosurgery (SRS).
A systematic review was conducted according to PRISMA guidelines using the PubMed, Scopus, Web of Science, Embase, and Cochrane Library databases. Search terms included chordoma and radiosurgery and their equivalent terms. Data on baseline characteristics, SRS details, and outcomes were extracted. The Joanna Briggs Institute checklist was used to assess risk of bias. A meta-analysis was performed on relevant variables.
A total of 33 eligible studies encompassing 714 patients with skull base chordomas were included. Most studies had a low risk of bias. Patients, predominantly male (57.37%) with a mean age of 46.54 years, exhibited a conventional chordoma subtype (74.77%) and primary lesions (77.91%), mainly in the clivus (98.04%). The mean lesion volume was 13.49 cm3, and 96.68% of patients had undergone prior surgical attempts. Gamma Knife radiosurgery (88.76%) was the predominant SRS method. Radiologically, 27.19% of patients experienced tumor regression, while 55.02% showed no signs of disease progression at the latest follow-up. Progression occurred after a mean of 48.02 months. Symptom improvement was noted in 27.98% of patients. Radiosurgery was associated with a relatively low overall adverse event rate (11.94%), mainly cranial nerve deficits (8.72%). Meta-regression revealed that age and primary lesion type influenced symptom improvement, while factors like extent of resection, radiotherapy, and SRS type affected adverse event rates.
This systematic review provides evidence on the safety and effectiveness of radiosurgery in the management of skull base chordomas. Local tumor control was achieved in the majority of patients treated with SRS. Various baseline characteristics and SRS features have been analyzed to identify modifying factors for each outcome to provide a framework for informed decision-making when managing these patients.
软骨肉瘤是一种罕见的原发性骨肿瘤，其治疗方法的文献资料有限。尽管切除术被认为是一线治疗方法，但并不总能提供充分的肿瘤控制。在这项系统评价中，作者旨在通过立体定向放射外科（SRS）来全面了解这些肿瘤的治疗方法。
根据 PRISMA 指南，使用 PubMed、Scopus、Web of Science、Embase 和 Cochrane Library 数据库进行系统评价。检索词包括软骨肉瘤和放射外科及其等效术语。提取基线特征、SRS 细节和结果数据。使用 Joanna Briggs 研究所清单评估偏倚风险。对相关变量进行荟萃分析。
共纳入 33 项符合条件的研究，涵盖 714 例颅底软骨肉瘤患者。大多数研究的偏倚风险较低。患者主要为男性（57.37%），平均年龄为 46.54 岁，表现为典型的软骨肉瘤亚型（74.77%）和原发性病变（77.91%），主要位于斜坡（98.04%）。平均病变体积为 13.49cm3，96.68%的患者曾接受过手术尝试。伽玛刀放射外科（88.76%）是主要的 SRS 方法。影像学上，27.19%的患者肿瘤有消退，55.02%的患者在最新随访时无疾病进展迹象。进展发生在平均 48.02 个月后。27.98%的患者症状改善。放射外科的总体不良事件发生率相对较低（11.94%），主要是颅神经损伤（8.72%）。Meta 回归显示，年龄和原发性病变类型影响症状改善，而切除范围、放疗和 SRS 类型等因素则影响不良事件发生率。
本系统评价提供了放射外科治疗颅底软骨肉瘤的安全性和有效性证据。大多数接受 SRS 治疗的患者实现了局部肿瘤控制。分析了各种基线特征和 SRS 特征，以确定每个结果的调节因素，为管理这些患者时做出知情决策提供框架。
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