Department of Neurosurgery, China National Clinical Research Center for Neurological Diseases, Beijing Tiantan Hospital, Capital Medical University, Fengtai District, Beijing, 100050, China.
Department of Orthopedics, Peking University Third Hospital, Haidian District, Beijing, 100191, China.
Neurosurg Rev. 2020 Oct;43(5):1315-1322. doi: 10.1007/s10143-019-01161-w. Epub 2019 Aug 17.
Chordoid gliomas are extremely rare entities, which are generally considered occurring exclusively in the third ventricle. Despite the low-grade histological grade, aggressive behaviors have been reported in literatures. Due to the low morbidity, the origins, clinical, and radiological features, management and prognosis are still yet to be well elucidated. We retrospectively reviewed the clinical profiles from a series of 6 patients with chordoid gliomas. All patients underwent surgical treatment, and the diagnoses were based on histopathological examinations. Magnetic resonance imaging (MRI) was performed perioperatively. Follow-up outcomes were presented. This case series consisted of three male and three female patients (age range 27-67 years; mean age 43.3 years). MRI results showed tumors in the third ventricle (4/6), temporal-parietal-occipital lobe involving the lateral ventricle (1/6), and cerebellar hemisphere (1/6). Three tumors were solid, and the others were cystic-solid. Hydrocephalus was present in one patient. The T1-weighted imaging showed hypo- to isointensity, and T2-weighted imaging showed iso- to hyperintensity; enhancement was homogeneous (4/6) or heterogeneous (2/6). Diffusion-weighted imaging showed no evidence of restricted diffusion. Magnetic resonance spectrum showed an elevated choline value and reduced N-acetylaspartate value. Gross total resection was achieved in all patients, and during an average follow-up period of 35.8 months, no recurrence was noted. Chordoid gliomas can occur outside the third ventricle with a great diagnostic challenge. The MRI characteristics suggest a low-grade tumor, and the accurate diagnosis depends on pathological criteria. Complete surgical resection is associated with a favorable outcome.
脊索样胶质瘤是一种极为罕见的肿瘤，通常被认为仅发生于第三脑室。尽管组织学分级为低级，但已有文献报道其具有侵袭性。由于发病率低，其起源、临床和影像学特征、治疗和预后仍有待阐明。我们回顾性分析了 6 例脊索样胶质瘤患者的临床资料。所有患者均接受了手术治疗，诊断基于组织病理学检查。术前行磁共振成像（MRI）检查。随访结果一并呈现。该病例系列包括 3 例男性和 3 例女性患者（年龄 27-67 岁；平均年龄 43.3 岁）。MRI 结果显示肿瘤位于第三脑室（4/6）、颞顶枕叶累及侧脑室（1/6）和小脑半球（1/6）。3 个肿瘤为实性，其余为囊实性。1 例患者存在脑积水。T1 加权成像呈低或等信号，T2 加权成像呈等或高信号；增强呈均匀（4/6）或不均匀（2/6）。弥散加权成像未见弥散受限。磁共振波谱显示胆碱值升高，N-乙酰天门冬氨酸值降低。所有患者均行肿瘤全切除，平均随访 35.8 个月，未见复发。脊索样胶质瘤可发生于第三脑室以外，诊断极具挑战性。MRI 特征提示为低度肿瘤，准确诊断依赖于病理学标准。完全手术切除与良好的预后相关。
