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[颅内神经节胶质瘤。20例患者的系列综述]

[Intracranial gangliogliomas. A review of a series of 20 patients].

作者信息

Gelabert-González M, Amo J M Santín, Arcos Algaba A, Serramito García R, Castro Bouzas D, Díaz Cabana L, Prieto González A, Aran Echabe E, Bandín Diéguez F J, Villa Fernández J, García Allut A

机构信息

Servicio de Neurocirugía, Departamento de Cirugía, Hospital Clínico Universitario de Santiago de Compostela, Santiago de Compostela, La Coruña, España.

出版信息

Neurologia. 2011 Sep;26(7):405-15. doi: 10.1016/j.nrl.2010.09.014. Epub 2010 Nov 18.

Abstract

INTRODUCTION

A ganglioglioma is a type of primary central nervous system low grade tumour composed of mixed populations of glial and neuroepithelial elements. They accounts for 0.4 to 2% of all intracranial tumours and appear more commonly in children and young adults. Seizures, which are the most important symptom in these tumours, improve significantly after surgical excision.

METHODS

Between 1995 and 2008, 20 patients with (12 adults and 8 children) with intracranial ganglioglioma were treated at our hospital. Clinical information obtained by chart review included sex, age at onset of symptoms, clinical history, results of neurological examination, tumour location, CT and MRI appearance, surgical results and follow-up. All patients underwent tumour resection and the extent of surgery was determined from the surgical reports and postoperative imaging studies.

RESULTS

The median age of patients was 26.4 years (range, 1-75 years), and the female to male ratio was 1.5:1. Except in one case, all patients had seizures with a median duration before diagnosis of 7.4 years (range 1-29). Seventeen tumours were located in the temporal lobe (9 right and 8 left). Macroscopically complete excision was performed in 17 patients and subtotal in the remaining 3. There were 4 cases of recurrence treated by surgery and radiotherapy being added in one case. The mean follow up was 8.5 years (range 22 months-14 years) and disease free survival at 5 years was 85% and an overall survival of 95%.

CONCLUSIONS

The seizures, which are the most frequent symptoms, significantly improved after surgical removal. Surgery is the first choice of therapy in these tumours, and in the presence of subtotal resection or tumour recurrence the best indication for treatment is repeat surgery. Radiotherapy should be reserved only for malignant forms.

摘要

引言

神经节胶质瘤是一种原发性中枢神经系统低级别肿瘤,由神经胶质和神经上皮成分混合组成。它们占所有颅内肿瘤的0.4%至2%,更常见于儿童和年轻人。癫痫发作是这些肿瘤最重要的症状,手术切除后会显著改善。

方法

1995年至2008年期间,我院治疗了20例颅内神经节胶质瘤患者(12例成人和8例儿童)。通过病历审查获得的临床信息包括性别、症状出现时的年龄、临床病史、神经学检查结果、肿瘤位置、CT和MRI表现、手术结果及随访情况。所有患者均接受了肿瘤切除术,手术范围根据手术报告和术后影像学检查确定。

结果

患者的中位年龄为26.4岁(范围1 - 75岁),男女比例为1.5:1。除1例患者外,所有患者均有癫痫发作,诊断前的中位病程为7.4年(范围1 - 29年)。17个肿瘤位于颞叶(右侧9个,左侧8个)。17例患者进行了肉眼下全切,其余3例为次全切除。有4例复发,其中1例加用了手术和放疗进行治疗。平均随访时间为8.5年(范围22个月 - 14年),5年无病生存率为85%,总生存率为95%。

结论

癫痫发作是最常见的症状,手术切除后显著改善。手术是这些肿瘤的首选治疗方法,在次全切除或肿瘤复发的情况下,最佳治疗指征是再次手术。放疗仅适用于恶性类型。

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