Department of Neurosurgery, Warren Alpert Medical School of Brown University, Providence, Rhode Island, USA; Computational Neurosciences Outcomes Center, Department of Neurosurgery, Brigham & Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
Computational Neurosciences Outcomes Center, Department of Neurosurgery, Brigham & Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
World Neurosurg. 2018 Jan;109:e676-e683. doi: 10.1016/j.wneu.2017.10.054. Epub 2017 Oct 20.
Chordoma that occurs primarily in the sella turcica is rare and presents unique treatment challenges.
The purpose of this study was to determine common features, treatment approaches, and survival characteristics of intrasellar chordoma.
Institutional databases, the SEER (Surveillance Epidemiology and End Results) database, and PubMed/EMBASE were queried for chordoma with a primarily intrasellar component. The SEER database was also queried for adult skull base chordoma. Patient-level data were extracted where available. Kaplan-Meier survival analyses were conducted.
Among 80 cases, the mean age at presentation was 55.6 (standard deviation, 15.9), with a female predominance (1.16:1.00). Patients experienced symptoms for a mean duration of 19.0 months, including cranial nerve deficits, hypopituitarism, and hyperprolactinemia. Among patients receiving treatment, 77.5% underwent surgery. In addition, less than half of the patients (n = 34, 47.3%) received adjuvant radiation therapy. The 5-year overall survival (OS) of intrasellar chordoma was 60.0% (standard error [SE], 6.9). Patients aged 40 years and younger had a 5-year OS of 80.8% (SE, 12.2), compared with patients older than 40 years, who had an OS of 55.4% (SE, 7.7) (Mantel-Cox, P = 0.044). Males experienced a lower 5-year OS (44.0; SE, 9.7) than did females (76.8; SE, 8.5), (Mantel-Cox, P = 0.003). Median OS was greater in patients with skull base chordoma than in patients with intrasellar chordoma (Mantel-Cox, P = 0.046).
Intrasellar chordoma presents frequently with visual disturbances and hyperprolactinemia and has a slightly higher incidence in females. Young age predicts a better prognosis. Intrasellar chordoma has a lower overall survival than has skull base chordoma.
主要发生在蝶鞍的脊索瘤较为罕见，且具有独特的治疗挑战。
本研究旨在确定鞍内脊索瘤的常见特征、治疗方法和生存特点。
通过机构数据库、SEER（监测、流行病学和最终结果）数据库和 PubMed/EMBASE 检索主要发生在鞍内的脊索瘤。SEER 数据库还检索了成人颅底脊索瘤。在有患者水平数据的情况下提取数据。进行 Kaplan-Meier 生存分析。
在 80 例病例中，患者的中位年龄为 55.6 岁（标准差 15.9），女性略多见（1.16:1.00）。患者的症状持续时间平均为 19.0 个月，包括颅神经功能障碍、垂体功能减退和高泌乳素血症。在接受治疗的患者中，77.5%接受了手术。此外，不到一半的患者（n=34，47.3%）接受了辅助放疗。鞍内脊索瘤的 5 年总生存率（OS）为 60.0%（标准误差[SE]，6.9）。40 岁及以下患者的 5 年 OS 为 80.8%（SE，12.2），而 40 岁以上患者的 OS 为 55.4%（SE，7.7）（Mantel-Cox，P=0.044）。男性的 5 年 OS（44.0；SE，9.7）低于女性（76.8；SE，8.5）（Mantel-Cox，P=0.003）。颅底脊索瘤患者的中位 OS 长于鞍内脊索瘤患者（Mantel-Cox，P=0.046）。
鞍内脊索瘤常表现为视力障碍和高泌乳素血症，女性发病率略高。年轻是预后较好的预测因素。鞍内脊索瘤的总生存率低于颅底脊索瘤。
