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结节性硬化症患者室管膜下巨细胞星形细胞瘤的手术治疗结果。

The outcome of surgical management of subependymal giant cell astrocytoma in tuberous sclerosis complex.

机构信息

Paediatric Neurology, University of Bristol, Bristol, UK.

出版信息

Eur J Paediatr Neurol. 2013 Jan;17(1):36-44. doi: 10.1016/j.ejpn.2012.10.005. Epub 2012 Nov 24.

Abstract

OBJECTIVES

The indications for surgery and outcomes of patients who underwent surgical removal of subependymal giant cell astrocytomas (SEGAs) in our institution between 2000 and 2011 were reviewed.

METHODS

We reviewed the clinical details of 16 patients with a diagnosis of Tuberous Sclerosis Complex (TSC) who underwent surgery for SEGA in Bristol since 2000. We collected information on age, sex, epilepsy history and cognitive status. We reviewed the indications for surgery, age at surgery, surgical approach, and the size and location of the lesions. We analysed mortality, completeness of tumour resection, intraoperative blood transfusion, shunt placements, and surgical complications.

RESULTS

13 patients had surgery due to hydrocephalus. Increasing size of SEGA without hydrocephalus was an indication for surgery in two patients, and in one patient, the SEGA was removed because of its size and location at initial scan. 13 patients had complete tumour resection. One patient had tumour recurrence. Hydrocephalus failed to resolve or reoccurred in four patients post operatively necessitating shunt insertion. The surgical approach was transcortical in 14 patients and transcallosal in two. There was zero mortality in this series. There were no reports of cognitive decline or worsening epilepsy following surgery.

CONCLUSION

Surgery is a safe and effective treatment for SEGA. It is the authors' view that surgery remains the most appropriate treatment strategy for SEGAs that are amenable to surgery. More work needs to be undertaken to assess prospectively the neurocognitive impact of surgery, and the relative advantages of different surgical approaches.

摘要

目的

回顾本机构 2000 年至 2011 年间手术切除室管膜下巨细胞星形细胞瘤(SEGA)患者的手术适应证和结果。

方法

我们回顾了自 2000 年以来在布里斯托尔因 SEGA 而接受手术的 16 名结节性硬化症(TSC)患者的临床详细资料。我们收集了年龄、性别、癫痫病史和认知状态的信息。我们回顾了手术适应证、手术年龄、手术入路、病变的大小和位置。我们分析了死亡率、肿瘤切除的完整性、术中输血、分流器放置和手术并发症。

结果

13 名患者因脑积水而接受手术。两名患者因 SEGA 无脑积水而增大,另有一名患者因初始扫描时 SEGA 的大小和位置而接受手术。13 名患者行肿瘤全切除。1 名患者肿瘤复发。4 名患者术后脑积水未缓解或再次发生,需要放置分流器。14 名患者采用皮质切开入路,2 名患者采用胼胝体切开入路。本系列无死亡病例。术后无认知能力下降或癫痫恶化的报告。

结论

手术是 SEGA 的安全有效治疗方法。作者认为,对于适合手术的 SEGA,手术仍然是最恰当的治疗策略。需要进一步开展工作,前瞻性评估手术对神经认知的影响,以及不同手术入路的相对优势。

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