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颅内脊索样胶质瘤:14 例临床、放射学和病理学研究。

Intracranial chordoid glioma: A clinical, radiological and pathological study of 14 cases.

机构信息

Department of Neurosurgery, China National Clinical Research Center for Neurological Diseases (NCRC-ND), Center of Brain Tumor, Beijing Institute for Brain Disorders, Beijing Key Laboratory of Brian Tumor, Beijing Tiantan Hospital, Capital Medical University, Beijing 100050, PR China.

Department of Critical Care Medicine, Beijing Tiantan Hospital, Capital Medical University, Beijing 100050, PR China.

出版信息

J Clin Neurosci. 2020 Oct;80:267-273. doi: 10.1016/j.jocn.2020.09.019. Epub 2020 Sep 18.

Abstract

BACKGROUND

Chordoid gliomas (CGs) are rare neuroepithelial tumors, which commonly arise from the anterior part of the third ventricle. Most studies on CGs included only one or two cases. To better understand the disease, we report 14 patients with pathologically confirmed CGs.

METHOD

The clinical characteristics, including radiological and histological examination, operative records, and prognoses were analyzed and reviewed.

RESULT

The case series included six male and eight female patients with an average age of 44.4 years. The most common preoperative symptom was headache (64.3%) and visual deterioration (57.1%). Radiological results showed that the third ventricle (12/14) was the most common site of the brain involved, and the lesions presented with solid (n = 9, 64.3%) or cystic-solid (n = 5, 35.7%) appearance. All patients were misdiagnosed as non-CG tumors. The operation approach was mainly determined by tumor location, thus trans-callosal approach (9/14) and trans-laminar terminalis approach were commonly used. Gross total resection (GTR) was achieved in all cases and none of them received any adjuvant therapy postoperatively. The most frequent postoperative complications were diabetes insipidus, electrolyte disturbance, hypopituitarism, cognitive dysfunction, and obstructive hydrocephalus. During an average follow-up period of 40.1 months, 2 cases (14.3%) were died of refractory hypopituitarism and pulmonary embolism, respectively. The preoperative symptoms and postoperative complications were all significantly improved in other 12 patients, and MRI showed no tumor recurrence.

CONCLUSION

According to our experience, we recommend GTR as the primary goal, which is associated with improved rates of tumor control and without increasing rates of postoperative complications.

摘要

背景

脊索样胶质瘤(CGs)是一种罕见的神经上皮肿瘤,通常发生于第三脑室前部。大多数 CGs 的研究仅包括一两个病例。为了更好地了解这种疾病,我们报告了 14 例经病理证实的 CGs 患者。

方法

分析和回顾了这些患者的临床特征,包括影像学和组织学检查、手术记录和预后。

结果

该病例系列包括 6 名男性和 8 名女性患者,平均年龄为 44.4 岁。最常见的术前症状是头痛(64.3%)和视力恶化(57.1%)。影像学结果显示,第三脑室(12/14)是最常见的受累脑区,病变表现为实性(9/14,64.3%)或囊实性(5/14,35.7%)。所有患者均误诊为非 CG 肿瘤。手术方法主要取决于肿瘤位置,因此常用经胼胝体入路(9/14)和经终板入路。所有患者均实现了全切除(GTR),术后均未接受任何辅助治疗。最常见的术后并发症是尿崩症、电解质紊乱、垂体功能减退、认知功能障碍和阻塞性脑积水。在平均 40.1 个月的随访期间,2 例(14.3%)患者分别因难治性垂体功能减退和肺栓塞死亡。其他 12 例患者的术前症状和术后并发症均明显改善,MRI 显示无肿瘤复发。

结论

根据我们的经验,我们建议将 GTR 作为主要目标,这与提高肿瘤控制率且不增加术后并发症发生率相关。

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