OBJECTIVE Clivus chordomas are semi-malignant, but infiltratively growing tumors. Currently, a widely-accepted treatment concept encompasses maximal, but safe, surgical resection and radiotherapy. Caused by the size and the tumor extension, different surgical approaches, especially in recurrent cases, might be necessary. METHODS Retrospective review of 50 patients on whom 70 surgeries were performed: 29 in primary and 41 in recurrent cases. Based on MRI images, all cases were asserted according to the size and the extension of the tumor. Used surgical approaches were evaluated. Postoperative complications, neurological function prior to and after the surgery, the extent of tumor resection on postoperative MR images were assessed and progression-free survival was calculated. RESULTS Tumor size was estimated as small (< 5 cm3) in 8, as medium (5-20 cm3) in 21, as large (20-100 cm3) in 17, and as giant (> 100 cm3) in 4 patients. Most frequently used surgical approaches in primary cases were the transsphenoidal one and midfacial degloving (51.7 % and 17.2 %, respectively). In recurrent cases, dependent on the tumor extension, transsphenoidal (21.9 %), retrosigmoidal (29.3 %), and pterional (19.5 %) approaches, as well as midfacial degloving (17.1 %) were used. Due to the vast tumor extension and infiltration, gross total or near total resection could be achieved in 12 patients (24 %), only. There was no mortality and no major complications in primary cases. In recurrences, however, postoperative hemorrhages and strokes emerged in 4.9 % and 7.1 %. Minor complications occurred in 17.1 % and were dominated by CSF leaks (12.2 %), both in primary in recurrent cases. While most cranial nerve impairments were caused by tumor infiltration of the cavernous sinus, and hence have not improved by treatment, the sixth nerve palsy as a consequence of tumor mass compression, could significantly be improved by surgery. Following surgery, patients were subjected to radiotherapy (68.9 % for primary cases, and 36.6 % for recurrences) mainly with carbon ions. Overall, 5-year progression-free survival was 44.7 %. CONCLUSION Caused by the heterogenous pattern of growth of clivus chordomas, surgical approaches should be chosen individually. Vast and infiltrative tumor extension constitute major limitations of surgical resection, and hence result in poor progression-frees survival.

中文翻译：

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斜坡脊索瘤：异质性肿瘤扩展需要适应的手术方法

目的 Clivus 脊索瘤是半恶性但浸润性生长的肿瘤。目前，广泛接受的治疗概念包括最大限度但安全的手术切除和放疗。由于大小和肿瘤扩展，可能需要不同的手术方法，特别是在复发病例中。方法 对 50 例患者进行了 70 次手术的回顾性研究：原发病例 29 例，复发病例 41 例。根据 MRI 图像，所有病例均根据肿瘤的大小和范围进行断言。评估使用的手术方法。评估术后并发症、手术前后的神经功能、术后 MR 图像上的肿瘤切除范围并计算无进展生存期。结果 肿瘤大小估计为 8 个小 (< 5 cm3)，中型 (5-20 cm3) 21 例，大型 (20-100 cm3) 17 例，巨型 (> 100 cm3) 4 例。在原发病例中最常用的手术方法是经蝶窦和面中部脱套（分别为 51.7 % 和 17.2 %）。在复发病例中，取决于肿瘤的扩展，使用经蝶窦（21.9 %）、乙状窦后（29.3 %）和翼点（19.5 %）入路，以及面中部脱套（17.1 %）。由于广泛的肿瘤扩展和浸润，仅 12 名患者 (24 %) 可以实现大体完全或接近完全切除。在原发病例中没有死亡和严重并发症。然而，在复发中，术后出血和中风的发生率为 4.9% 和 7.1%。轻微并发症发生率为 17.1%，主要是脑脊液渗漏 (12.2%)，两者都是复发病例的原发性并发症。虽然大多数颅神经损伤是由海绵窦的肿瘤浸润引起的，因此没有通过治疗得到改善，但由于肿瘤块压迫导致的第六神经麻痹，可以通过手术得到显着改善。手术后，患者接受主要使用碳离子的放射治疗（原发病例为 68.9%，复发病例为 36.6%）。总体而言，5 年无进展生存率为 44.7%。结论 由于斜坡脊索瘤的异质性生长模式，手术入路应个体化选择。巨大的浸润性肿瘤扩展构成了手术切除的主要限制，因此导致无进展生存率较差。由于肿瘤块压迫导致的第六神经麻痹，可以通过手术显着改善。手术后，患者接受主要使用碳离子的放射治疗（原发病例为 68.9%，复发病例为 36.6%）。总体而言，5 年无进展生存率为 44.7%。结论 由于斜坡脊索瘤的异质性生长模式，手术入路应个体化选择。巨大的浸润性肿瘤扩展构成了手术切除的主要限制，因此导致无进展生存率较差。由于肿瘤块压迫导致的第六神经麻痹，可以通过手术显着改善。手术后，患者接受主要使用碳离子的放射治疗（原发病例为 68.9%，复发病例为 36.6%）。总体而言，5 年无进展生存率为 44.7%。结论 由于斜坡脊索瘤的异质性生长模式，手术入路应个体化选择。巨大的浸润性肿瘤扩展构成了手术切除的主要限制，因此导致无进展生存率较差。结论 由于斜坡脊索瘤的异质性生长模式，手术入路应个体化选择。巨大的浸润性肿瘤扩展构成了手术切除的主要限制，因此导致无进展生存率较差。结论 由于斜坡脊索瘤的异质性生长模式，手术入路应个体化选择。巨大的浸润性肿瘤扩展构成了手术切除的主要限制，因此导致无进展生存率较差。