Departments of1Neurological Surgery.
2Radiation Oncology.
J Neurosurg. 2023 Oct 13;140(4):920-928. doi: 10.3171/2023.7.JNS231196. Print 2024 Apr 1.
The objective of this study was to clarify the detailed clinical course of recurrent clival chordoma and the outcomes of each treatment modality.
A single-center retrospective analysis was conducted on patients seen for recurrent clival chordoma. The cohort was identified from those who underwent surgery, stereotactic radiosurgery, or proton therapy at the authors' institution between 1990 and 2022.
A total of 95 recurrences in 40 patients with a median (interquartile range [IQR]) follow-up of 43 (18-79) months were identified. The median (IQR) age at the time of diagnosis was 48 (36-62) years, and 55% of patients were male. Twenty-three patients were treated with surgery followed by adjuvant radiation before the first recurrence. The median (range) number of recurrences per patient was 2 (1-8), and the median (IQR) time to the first recurrence was 29 (9-51) months. The recurrences were treated with one or more of the following therapies: surgery, radiation, systemic therapy, and laser interstitial thermal therapy (LITT). Surgery was performed for 44 recurrences in 25 patients. Radiation was used to treat 42 recurrences in 28 patients. Patients with recurrences treated with surgery plus radiation had the longest progression-free survival (PFS) (median [95% CI] overall survival [OS] 120 [0-245] months, p < 0.01, log-rank test). Patients with recurrences but without prior radiation had longer PFS than those patients with prior radiation. The median (95% CI) OS after the first recurrence was 68 (54-82) months, 5-year OS after the first recurrence was 48%, and 10-year OS was 27%. Multivariate Cox regression analysis showed that mortality after the first recurrence was significantly associated with no adjuvant radiation (HR 0.149, 95% CI 0.038-0.59, p = 0.0067), older age at the time of the first recurrence (HR 1.04, 95% CI 1.01-1.08, p = 0.021), and total number of recurrences (p = 0.032). Seven patients received systemic therapy, and the median (95% CI) OS of these patients since initiation of systemic therapy was 31 (11-51) months. Imatinib and/or nivolumab were used in 6 patients (15%). One patient (3%) was treated with LITT for his fourth recurrence.
Despite the aggressive nature of recurrent chordoma, 14 of 29 patients (48%) survived for more than 5 years after the initial recurrence using combined therapies. Multiple treatment options may contribute to the long-term survival of patients with this intractable tumor.
本研究旨在阐明复发性颅底脊索瘤的详细临床过程以及每种治疗方式的结果。
对在作者所在机构于 1990 年至 2022 年间接受手术、立体定向放射外科或质子治疗的复发性颅底脊索瘤患者进行单中心回顾性分析。
共发现 40 名患者的 95 例复发，中位（四分位距 [IQR]）随访时间为 43（18-79）个月。诊断时的中位（IQR）年龄为 48（36-62）岁，55%的患者为男性。23 名患者在首次复发前接受了手术加辅助放疗。每位患者的中位（范围）复发次数为 2（1-8），首次复发的中位（IQR）时间为 29（9-51）个月。复发病例采用以下一种或多种治疗方法：手术、放疗、系统治疗和激光间质热疗（LITT）。25 名患者的 44 例复发采用了手术治疗。28 名患者的 42 例复发采用了放疗。接受手术加放疗治疗的患者无进展生存期（PFS）最长（中位[95%CI]总生存期[OS]120[0-245]个月，p<0.01，对数秩检验）。没有接受过放疗的复发病例患者的 PFS 长于接受过放疗的患者。首次复发后的中位（95%CI）OS 为 68（54-82）个月，首次复发后 5 年 OS 为 48%，10 年 OS 为 27%。多变量 Cox 回归分析显示，首次复发后的死亡率与无辅助放疗显著相关（HR 0.149，95%CI 0.038-0.59，p=0.0067）、首次复发时年龄较大（HR 1.04，95%CI 1.01-1.08，p=0.021）和复发总数（p=0.032）。7 名患者接受了系统治疗，自开始系统治疗以来，这些患者的中位（95%CI）OS 为 31（11-51）个月。6 名患者（15%）使用了伊马替尼和/或纳武单抗。1 名患者（3%）因第四次复发接受了 LITT 治疗。
尽管复发性脊索瘤具有侵袭性，但 29 名患者中有 14 名（48%）在首次复发后使用联合治疗存活超过 5 年。多种治疗选择可能有助于长期生存患者的这种难治性肿瘤。
