Abstract：
This study aimed to provide data on extended outcomes in primary clival chordomas, focusing on progression-free survival (PFS) and overall survival (OS).
A retrospective single-center analysis was conducted on patients with clival chordoma treated between 1987 and 2022 using surgery, stereotactic radiosurgery, or proton radiation therapy (PRT).
The study included 100 patients (median age 44 years, 51% male). Surgery was performed using the endoscopic endonasal approach in 71 patients (71%). Gross-total resection (GTR) or near-total resection (NTR) was attained in 39 patients (39%). Postoperatively, new cranial nerve deficits occurred in 7%, CSF leak in 4%, and meningitis in none of the patients. Radiation therapy was performed in 79 patients (79%), with PRT in 50 patients (50%) as the primary treatment. During the median follow-up period of 73 (interquartile range [IQR] 38-132) months, 41 recurrences (41%) and 31 deaths (31%) were confirmed. Patients with GTR/NTR had a median PFS of 41 (IQR 24-70) months. Patients with subtotal resection or biopsy had a median PFS of 38 (IQR 16-97) months. The median PFS of patients who received radiation therapy was 43 (IQR 26-86) months, while that of patients who did not receive radiation therapy was 18 (IQR 5-62) months. The Kaplan-Meier method showed that patients with GTR/NTR (p = 0.007) and those who received radiation therapy (p < 0.001) had longer PFS than their counterparts. The PFS rates following primary treatment at 5, 10, 15, and 20 years were 51%, 25%, 17%, and 7%, respectively. The OS rates at the same intervals were 84%, 60%, 42%, and 34%, respectively. Multivariate Cox regression analysis showed that age < 44 years (p = 0.02), greater extent of resection (EOR; p = 0.03), and radiation therapy (p < 0.001) were associated with lower recurrence rates. Another multivariate analysis showed that age < 44 years (p = 0.01), greater EOR (p = 0.04), and freedom from recurrence (p = 0.02) were associated with lower mortality rates. Regarding pathology data, brachyury was positive in 98%, pan-cytokeratin in 93%, epithelial membrane antigen in 85%, and S100 in 74%. No immunohistochemical markers were associated with recurrence.
In this study, younger age, maximal safe resection, and radiation therapy were important factors for longer PFS in patients with primary clival chordomas. Preventing recurrences played a crucial role in achieving longer OS.
A retrospective single-center analysis was conducted on patients with clival chordoma treated between 1987 and 2022 using surgery, stereotactic radiosurgery, or proton radiation therapy (PRT).
The study included 100 patients (median age 44 years, 51% male). Surgery was performed using the endoscopic endonasal approach in 71 patients (71%). Gross-total resection (GTR) or near-total resection (NTR) was attained in 39 patients (39%). Postoperatively, new cranial nerve deficits occurred in 7%, CSF leak in 4%, and meningitis in none of the patients. Radiation therapy was performed in 79 patients (79%), with PRT in 50 patients (50%) as the primary treatment. During the median follow-up period of 73 (interquartile range [IQR] 38-132) months, 41 recurrences (41%) and 31 deaths (31%) were confirmed. Patients with GTR/NTR had a median PFS of 41 (IQR 24-70) months. Patients with subtotal resection or biopsy had a median PFS of 38 (IQR 16-97) months. The median PFS of patients who received radiation therapy was 43 (IQR 26-86) months, while that of patients who did not receive radiation therapy was 18 (IQR 5-62) months. The Kaplan-Meier method showed that patients with GTR/NTR (p = 0.007) and those who received radiation therapy (p < 0.001) had longer PFS than their counterparts. The PFS rates following primary treatment at 5, 10, 15, and 20 years were 51%, 25%, 17%, and 7%, respectively. The OS rates at the same intervals were 84%, 60%, 42%, and 34%, respectively. Multivariate Cox regression analysis showed that age < 44 years (p = 0.02), greater extent of resection (EOR; p = 0.03), and radiation therapy (p < 0.001) were associated with lower recurrence rates. Another multivariate analysis showed that age < 44 years (p = 0.01), greater EOR (p = 0.04), and freedom from recurrence (p = 0.02) were associated with lower mortality rates. Regarding pathology data, brachyury was positive in 98%, pan-cytokeratin in 93%, epithelial membrane antigen in 85%, and S100 in 74%. No immunohistochemical markers were associated with recurrence.
In this study, younger age, maximal safe resection, and radiation therapy were important factors for longer PFS in patients with primary clival chordomas. Preventing recurrences played a crucial role in achieving longer OS.
摘要：
目的：本研究旨在提供原发性斜坡脊索瘤的延长结局数据，关注无进展生存期（PFS）和总生存期（OS）。
方法：对1987年至2022年间使用手术治疗的斜坡脊索瘤患者进行了回顾性单中心分析，立体定向放射外科,或质子放射治疗（PRT）。
结果：该研究包括100名患者（中位年龄44岁，51%男性）。71例患者（71％）使用鼻内镜入路进行手术。39例患者（39％）获得了大体全切除（GTR）或接近全切除（NTR）。术后,新的颅神经缺陷发生在7%，脑脊液泄漏4%，没有一个病人出现脑膜炎。79例患者（79%）接受放射治疗，以PRT50例（50％）为主要治疗方法。在73个月（四分位距[IQR]38-132）的中位随访期间，确诊41例复发（41%）和31例死亡（31%）。GTR/NTR患者的中位PFS为41(IQR24-70)个月。进行次全切除或活检的患者的中位PFS为38个月（IQR16-97）。接受放射治疗的患者的中位PFS为43（IQR26-86）个月，而未接受放射治疗的患者为18个月（IQR5-62）。Kaplan-Meier方法显示，GTR/NTR患者（p=0.007）和接受放射治疗的患者（p<0.001）的PFS比同行更长。初级治疗后5、10、15和20年的PFS率为51％，25%,17%,7%,分别。相同时间间隔的OS率为84%，60%,42%,34%,分别。多因素Cox回归分析显示年龄<44岁(p=0.02),更大程度的切除（EOR；p=0.03），和放射治疗（p<0.001）与较低的复发率相关。另一个多变量分析表明，年龄<44岁(p=0.01)，EOR更大（p=0.04），无复发(p=0.02)与较低的死亡率相关.关于病理学数据，98%的brachyury为阳性，泛细胞角蛋白占93%，85％的上皮膜抗原，和S100在74%。无免疫组织化学标记与复发相关。
结论：在这项研究中，年龄较小,最大安全切除,放射治疗是原发性斜坡脊索瘤患者PFS延长的重要因素。防止复发在实现更长的操作系统中起着至关重要的作用。
方法：对1987年至2022年间使用手术治疗的斜坡脊索瘤患者进行了回顾性单中心分析，立体定向放射外科,或质子放射治疗（PRT）。
结果：该研究包括100名患者（中位年龄44岁，51%男性）。71例患者（71％）使用鼻内镜入路进行手术。39例患者（39％）获得了大体全切除（GTR）或接近全切除（NTR）。术后,新的颅神经缺陷发生在7%，脑脊液泄漏4%，没有一个病人出现脑膜炎。79例患者（79%）接受放射治疗，以PRT50例（50％）为主要治疗方法。在73个月（四分位距[IQR]38-132）的中位随访期间，确诊41例复发（41%）和31例死亡（31%）。GTR/NTR患者的中位PFS为41(IQR24-70)个月。进行次全切除或活检的患者的中位PFS为38个月（IQR16-97）。接受放射治疗的患者的中位PFS为43（IQR26-86）个月，而未接受放射治疗的患者为18个月（IQR5-62）。Kaplan-Meier方法显示，GTR/NTR患者（p=0.007）和接受放射治疗的患者（p<0.001）的PFS比同行更长。初级治疗后5、10、15和20年的PFS率为51％，25%,17%,7%,分别。相同时间间隔的OS率为84%，60%,42%,34%,分别。多因素Cox回归分析显示年龄<44岁(p=0.02),更大程度的切除（EOR；p=0.03），和放射治疗（p<0.001）与较低的复发率相关。另一个多变量分析表明，年龄<44岁(p=0.01)，EOR更大（p=0.04），无复发(p=0.02)与较低的死亡率相关.关于病理学数据，98%的brachyury为阳性，泛细胞角蛋白占93%，85％的上皮膜抗原，和S100在74%。无免疫组织化学标记与复发相关。
结论：在这项研究中，年龄较小,最大安全切除,放射治疗是原发性斜坡脊索瘤患者PFS延长的重要因素。防止复发在实现更长的操作系统中起着至关重要的作用。
