儿科后窝外侧脑膜瘤

IF 0.7 4区 医学 Q4 NEUROSCIENCES
Pablo Miranda-Lloret, Estela Plaza-Ramírez, Juan Antonio Simal-Julián, Giovanni Pancucci, Adela Cañete, Alejandro Montoya-Filardi, Gemma Llavador
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引用次数: 0

摘要

背景和目的外侧型后窝外胚瘤在临床和病理上都是一种定义明确的亚型肿瘤,预后较差。其发病率较低,手术治疗具有挑战性。本研究的目的是回顾我们对侧位后窝外胚窦瘤的研究,并将我们的研究结果与之前的研究结果进行比较。材料和方法在过去 10 年中,我们儿科共手术治疗了 30 例外胚窦瘤患者,其中 7 例为侧位型后窝外胚窦瘤。结果患者平均年龄为 3.75 岁。六例患者伴有脑积水。确诊时的平均肿瘤体积为 61 毫升。六名患者实现了完全切除,一名患者接近完全切除。五名患者暂时需要进行胃造口术和气管造口术。平均随访时间为 58 个月。在此期间,一名患者病情恶化,最终死亡。四例脑积水患者需要进行脑室腹腔 CSF 分流术,两例患者进行了第三脑室造口术。在最后的随访中,4 名患者生活正常,2 名患者根据兰斯基量表显示有轻度限制。与下颅神经功能障碍相关的神经功能缺损很常见,但都是一过性的。对这些肿瘤进行更深入的基因鉴定可能会发现风险因素,为分层辅助治疗提供指导。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Ependimomas de fosa posterior de tipo lateral en la edad pediátrica

Background and aims

Lateral-type posterior fossa ependymomas are a well-defined subtype of tumors both clinically and pathologically, with a poor prognosis. Their incidence is low and surgical management is challenging. The objective of the present work is to review our series of lateral-tye posterior fossa ependymomas and compare our results with those of previous series.

Materials and methods

Among 30 cases of ependymoma operated in our pediatric department in the last 10 years, we identified seven cases of lateral-type posterior fossa ependymomas. We then performed a retrospective, descriptive study.

Results

Mean age of our patients was 3.75 years. Six cases presented with hydrocephalus. Mean tumor volume at diagnosis was 61 cc. A complete resection was achieved in six cases and a near-total resection in one patient. Five patients transiently required a gastrostomy and a tracheostomy. Mean follow-up was 58 months. One case progressed along this period and eventually died. Four cases of hydrocephalus required a ventriculoperitoneal CSF shunt and two were managed with a third ventriculostomy. At last follow-up four patients carried a normal life and two displayed a mild restriction according to Lansky's scale.

Conclusions

The aim of surgical treatment in lateral-type posterior fossa ependymomas is complete resection. Neurological deficits associated to lower cranial nerve dysfunction are common but transient. Deeper genetic characterization of these tumors may identify risk factors that guide stratification of adjuvant therapies.

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来源期刊
Neurocirugia
Neurocirugia 医学-神经科学
CiteScore
1.30
自引率
0.00%
发文量
67
审稿时长
60 days
期刊介绍: Neurocirugía is the official Journal of the Spanish Society of Neurosurgery (SENEC). It is published every 2 months (6 issues per year). Neurocirugía will consider for publication, original clinical and experimental scientific works associated with neurosurgery and other related neurological sciences. All manuscripts are submitted for review by experts in the field (peer review) and are carried out anonymously (double blind). The Journal accepts works written in Spanish or English.
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