Lee Sarah, Teferi Nahom, Vivanco-Suarez Juan, Chowdhury Ajmain, Glennon Stephen, Kato Kyle, Matern Tyson, Eschbacher Kathryn L, Petronek Michael, Hitchon Patrick
Department of Neurosurgery, University of Iowa Carver, College of Medicine, Iowa City, IA, United States.
University of Iowa Carver, College of Medicine, Iowa City, IA, United States.
N Am Spine Soc J. 2024 Nov 4;20:100569. doi: 10.1016/j.xnsj.2024.100569. eCollection 2024 Dec.
Chordomas are rare, slow growing, locally aggressive malignant bone tumors that arise from remnants of the embryonic notochord with variable presenting symptoms depending on tumor location.
All patients with craniospinal chordoma managed at our institution between 1982 and 2023 were retrospectively reviewed. Demographics, tumor characteristics, clinical course and treatment, and long-term neurological and survival outcomes were collected. Adjuvant radiotherapy (RT) was stratified into standard dose fractionated radiotherapy (standard XRT) for doses of 50 to 60 Gy at 1.8 Gy fractions or high dose hyperfractionated stereotactic radiotherapy (HD-FSRT) for doses of 60 to 81 Gy at 1.2-1.5 Gy fractions per treatment. Descriptive statistics, univariate analysis, Log-rank test, and Kaplan-Meier survival analysis were performed.
A total of 37 patients were included in our cohort (mean age 46.0 ± 20.8 years; 22 male). Clival chordomas accounted for the majority of patients (56.8%), followed by vertebral (27%) and sacral (10.8%) chordomas. Thirty-five patients (94.6%) underwent gross total resection (GTR) or subtotal resection (STR), and 2 patients underwent excisional biopsy only. Postoperatively, functional status trended towards improvement (KPS: Preop- 80 [range 40-100] vs. Post op- 90 [60-100], p = .0911) and all patients either maintained or improved their neurological function. Median overall survival (OS) after diagnosis was 16.5 years. Age < 65, clival tumor location, post-operative Frankel grade E, and administration of adjuvant RT following initial STR significantly improved OS. OS of GTR patients was not significantly affected by adjuvant RT treatment.
Our results show the best long-term survival outcomes for chordoma patients undergoing GTR of tumor tissue. Higher postoperative neurological function was significantly associated with OS, highlighting the importance of maximal but safe total tumor resection. Moreover, adjuvant RT improved long-term survival for patients that underwent STR but had no effect on survival outcomes for patients that underwent GTR.
脊索瘤是一种罕见的、生长缓慢的、具有局部侵袭性的恶性骨肿瘤，起源于胚胎脊索的残余组织，其症状因肿瘤位置而异。
对1982年至2023年在本机构接受治疗的所有颅脊索瘤患者进行回顾性研究。收集患者的人口统计学资料、肿瘤特征、临床病程及治疗情况，以及长期神经功能和生存结果。辅助放疗（RT）分为标准剂量分割放疗（标准XRT），剂量为50至60 Gy，每次分割剂量为1.8 Gy；或高剂量超分割立体定向放疗（HD-FSRT），剂量为60至81 Gy，每次治疗分割剂量为1.2 - 1.5 Gy。进行描述性统计、单因素分析、对数秩检验和Kaplan-Meier生存分析。
本队列共纳入37例患者（平均年龄46.0±岁；男性22例）。斜坡脊索瘤患者占大多数（56.8%），其次是脊椎（27%）和骶骨（10.8%）脊索瘤。35例患者（94.6%）接受了全切除（GTR）或次全切除（STR），2例患者仅接受了切除活检。术后，功能状态呈改善趋势（KPS：术前80[范围40 - 100] vs.术后90[60 - 100]，p = 0.0911），所有患者的神经功能均得以维持或改善。诊断后的中位总生存期（OS）为16.5年。年龄<65岁、斜坡肿瘤位置、术后Frankel分级E以及初次STR后给予辅助放疗显著改善了OS。GTR患者的OS不受辅助放疗治疗的显著影响。
我们的结果显示，接受肿瘤组织GTR的脊索瘤患者具有最佳的长期生存结果。术后较高的神经功能与OS显著相关，突出了最大程度但安全地切除全部肿瘤的重要性。此外，辅助放疗改善了接受STR患者的长期生存，但对接受GTR患者的生存结果没有影响。
