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伪装成前庭神经鞘瘤的桥小脑角间变性节细胞胶质瘤:罕见病例

Cerebellopontine Angle Anaplastic Ganglioglioma Masquerading as Vestibular Schwannoma: Unusual Entity.

作者信息

Dutta Gautam, Singh Daljit, Saran Ravindra K, Singh Hukum, Srivastava Arvind K, Jagetia Anita

机构信息

Department of Neuro-Surgery, Govind Ballabh Pant Institute of Postgraduate Medical Education and Research, New Delhi, India.

Department of Neuro-Surgery, Govind Ballabh Pant Institute of Postgraduate Medical Education and Research, New Delhi, India.

出版信息

World Neurosurg. 2018 Sep;117:221-224. doi: 10.1016/j.wneu.2018.06.054. Epub 2018 Jun 19.

Abstract

BACKGROUND

Anaplastic gangliogliomas at the cerebellopontine angle (CPA) are exceptionally rare with only a few reported cases in published literature. These are composed of atypical ganglion cells and astrocytes accounting for nearly 1% of all central nervous system tumors. The authors report the case of anaplastic ganglioglioma in an adult patient presenting as a CPA mass lesion. The presentation was bizarre, and the diagnosis was possible only after histopathologic examination of the resected mass.

CASE DESCRIPTION

A 32-year-old lady presented with complaints of gradually progressive hearing loss and tinnitus in her right ear along with headache and ataxia. Neurologic evaluation revealed right-sided sensorineural hearing loss and truncal ataxia. Magnetic resonance imaging of the brain was suggestive of a well-defined, contrast-enhancing, solid-cystic mass at the CPA cistern that extended into the porus acusticus, causing enlargement of the internal acoustic meatus. The features were suggestive of vestibular schwannoma of the CPA.

CONCLUSIONS

This unusual appearance of anaplastic ganglioglioma has not been previously described. Neurosurgeons need to keep in mind the possibility of anaplastic gangliogliomas in the differential diagnosis of CPA lesions.

摘要

背景

小脑脑桥角(CPA)间变性节细胞胶质瘤极为罕见,在已发表的文献中仅有少数病例报道。这些肿瘤由非典型神经节细胞和星形胶质细胞组成,约占所有中枢神经系统肿瘤的1%。作者报告了一例成年患者表现为CPA肿块病变的间变性节细胞胶质瘤病例。其临床表现奇特,仅在对切除肿块进行组织病理学检查后才得以确诊。

病例描述

一名32岁女性患者,主诉右耳听力逐渐减退、耳鸣,伴有头痛和共济失调。神经系统评估显示右侧感音神经性听力损失和躯干性共济失调。脑部磁共振成像提示CPA脑池内有一个边界清晰、有强化的实性囊性肿块,延伸至内耳道,导致内耳道扩大。这些特征提示CPA区前庭神经鞘瘤。

结论

间变性节细胞胶质瘤的这种不寻常表现此前尚未见报道。神经外科医生在CPA病变的鉴别诊断中需考虑到间变性节细胞胶质瘤的可能性。

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