Ependymomas occurring in the upper cervical spinal cord (above the level of the C4 segment) are rare entities with great therapeutic challenges. This study was aimed to investigate the clinicoradiological characteristics and the prognosis in a large cohort of upper cervical ependymomas from a single institution. This retrospective study enrolled 155 patients with primary ependymomas in the upper cervical spinal cord. The pre- and post-operative clinical and magnetic resonance imaging profiles were collected. The neurological outcomes and survival events were evaluated, and potential independent risk factors were analyzed. There were 82 females and 73 males, with an average age of 43.1 ± 11.3 years. Immediately post-operatively, 118 (76.1%) patients experienced neurological deterioration and 32 (20.7%) patients remained unchanged. Three months after surgery, 61 (39.4%) patients showed deteriorated neurological functions compared to the pre-operative baseline levels. After an average follow-up period of 56.0 ± 24.7 months, the neurological functions were worse than the baseline status in 37 (23.9%) patients and improved in 33 (21.3%) patients, respectively. Logistic regression analysis identified that lower age (≤ 42 years) and lower pre-operative MMS (I–II) were independent protective factors for predicting favorable neurological functions. Multivariate Cox regression analysis revealed that incomplete resection was the only independent risk factor associated with a shorter progression-free survival. Age and pre-operative functional status affect the long-term neurological outcomes, and incomplete resection was associated with a shorter survival. Our findings indicate that gross total resection should be the goal of surgical treatment of upper cervical ependymomas.

发生在上颈脊髓（高于C4段的水平）的室间隔膜瘤是罕见的实体，面临巨大的治疗挑战。这项研究的目的是调查来自单个机构的大量上颈室室管膜瘤的临床放射学特征和预后。这项回顾性研究招募了155例上颈脊髓原发性室间隔瘤患者。收集术前和术后的临床和磁共振成像图。评估了神经学结果和生存事件，并分析了潜在的独立危险因素。女性82例，男性73例，平均年龄43.1±11.3岁。术后立即有118例（76.1％）的患者出现神经功能恶化，而32例（20.7％）的患者保持不变。术后三个月，与术前基线水平相比，有61名（39.4％）患者的神经功能恶化。在56.0±24.7个月的平均随访期后，分别有37例（23.9％）的患者的神经功能低于基线状态，而33例（21.3％）的患者的神经功能改善了。Logistic回归分析表明，较低的年龄（≤42岁）和较低的术前MMS（I–II）是预测良好神经功能的独立保护因素。多变量Cox回归分析显示，不完全切除是与无进展生存期较短相关的唯一独立危险因素。年龄和术前功能状态会影响长期的神经系统结局，不完全切除会导致生存期缩短。