Department of Neurosurgery, Tongji Hospital, Huazhong University of Science and Technology, Wuhan, People's Republic of China.
Department of Neurology, Tongji Hospital, Huazhong University of Science and Technology, Wuhan, People's Republic of China.
World Neurosurg. 2014 May-Jun;81(5-6):790-7. doi: 10.1016/j.wneu.2013.01.010. Epub 2013 Jan 5.
To analyze the clinical characteristics, immunohistochemistry, and treatment outcomes for skull base chordomas and the correlation between extent of resection/pathology type and outcomes.
The clinical materials of 77 consecutive patients with skull base chordomas were analyzed retrospectively. Follow-up data were available in 66 patients, ranging from 6 to 142 months (mean, 59.6 months). Outcome in survival was assessed by the overall survival (OS) and progression-free survival (PFS). Outcome in function was evaluated by Karnofsky performance score.
Total or near-total tumor resection was achieved in 25 cases (32.5%), subtotal in 32 cases (47.6%), and partial resection in 9 cases (11.7%). Gamma knife radiosurgery was used in 22 patients (33.3%). Forty-two of the 77 patients had immunohistochemistry results and the rates of positive staining for cytokeratin, epithelial membrane antigen, vimentin, S-100 were 100%, 92.9%, 83.3%, and 88.1%, respectively. The PFS rates at 3, 5, and 8 years were 82.6%, 45.0%, and 18.2%, respectively. The OS rates at 3, 5, and 8 years were 89.2%, 70.9%, and 45.5 %, respectively. Less tumor resection and dedifferentiated pathology were risk factors for worse OS and PFS (P < 0.05). Among the 43 currently surviving patients, the mean Karnofsky performance score before the surgery and at the last follow-up were 87.3 and 82.4, respectively.
Aggressive surgical resection should be performed for skull base chordomas, considering certain postoperative functional status. Immunohistochemical study is helpful in differential diagnosis. The combination of aggressive surgical resection and gamma knife radiosurgery for skull base chordomas may obtain favorable outcomes.
分析颅底脊索瘤的临床特征、免疫组织化学特征及治疗效果，并探讨肿瘤切除程度/病理类型与预后的相关性。
回顾性分析 77 例颅底脊索瘤患者的临床资料。66 例患者获得随访，随访时间为 6~142 个月，平均随访时间为 59.6 个月。采用总生存（OS）和无进展生存（PFS）评估生存预后，采用 Karnofsky 评分评估功能预后。
25 例（32.5%）患者达到全切除或近全切除，32 例（47.6%）患者达到次全切除，9 例（11.7%）患者达到部分切除。22 例（33.3%）患者接受了伽玛刀放射外科治疗。42 例患者进行了免疫组织化学检测，细胞角蛋白、上皮膜抗原、波形蛋白、S-100 阳性表达率分别为 100%、92.9%、83.3%和 88.1%。3、5、8 年的 PFS 率分别为 82.6%、45.0%和 18.2%，OS 率分别为 89.2%、70.9%和 45.5%。肿瘤切除程度较低和病理分级较差是影响 OS 和 PFS 的危险因素（P<0.05）。在 43 例目前存活的患者中，手术前和末次随访时的 Karnofsky 评分分别为 87.3 和 82.4。
考虑到术后一定的功能状态，颅底脊索瘤应行积极的手术切除。免疫组织化学研究有助于鉴别诊断。积极的手术切除联合伽玛刀放射外科治疗颅底脊索瘤可能获得良好的治疗效果。
