成人脑实质内神经鞘瘤表现为顶枕叶脑室周围病变:诊断和手术挑战

IF 2.5 Q3 CLINICAL NEUROLOGY
Christin Berjaoui , Ali Akil , Hussein Akil , Paul Hage , Joseph Mantoura
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引用次数: 0

摘要

摘要实质内神经鞘瘤(IS)极为罕见,在颅内神经鞘瘤中所占比例不到1%。迄今为止,文献中报道了大约150例,其中只有10例涉及顶枕区。与前庭神经鞘瘤不同,IS与颅神经缺乏明显的关联,常表现为高级别胶质瘤或转移性肿瘤,导致诊断不确定性。脑实质内神经鞘瘤的极端罕见和不典型的顶枕位置如何导致诊断挑战和潜在的误诊?材料与方法在PubMed、MEDLINE和谷歌Scholar上进行综合文献检索,关键词为“实质内神经鞘瘤”。文献共使用了150例先前报道的病例。结果我们报告一位56岁女性病患,表现为进行性构音障碍、眩晕、共济失调及右侧偏瘫。MRI显示左侧顶枕大脑室周围病变,伴实性和囊性混合成分,病灶周围明显水肿。鉴于进行性神经功能恶化,我们对病变进行了大体全切除。术中,肿瘤表现出坚固、边界清晰的形态。组织病理学检查证实了神经鞘瘤的诊断,这是一个在这个位置非常罕见的发现。结论本病例强调了IS的诊断挑战和手术切除对获得良好神经预后的重要性。鉴于其与侵袭性肿瘤的放射学重叠,先进的神经影像学和仔细的手术管理对于优化结果至关重要。提高对这种罕见病理的认识有助于早期识别,防止误诊,并指导有效的神经外科策略。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Intraparenchymal schwannoma in an adult presenting as a periventricular lesion of the parieto-occipital lobes: Diagnostic and surgical challenges

Introduction

Intraparenchymal schwannomas (IS) are exceedingly rare, constituting less than 1 % of all intracranial schwannomas. To date, approximately 150 cases have been reported in the literature, with only 10 involving the parieto-occipital region. Unlike vestibular schwannomas, IS lacks an apparent association with cranial nerves and often mimics high-grade gliomas or metastatic tumors, leading to diagnostic uncertainty.

Research question

How does the extreme rarity and atypical parieto-occipital location of intraparenchymal schwannomas contribute to diagnostic challenges and potential misdiagnosis?

Materials and methods

A comprehensive literature search was conducted using PubMed, MEDLINE, and Google Scholar with the keyword “Intraparenchymal Schwannoma”. A total of 150 previously reported cases were used for the literature.

Results

We report the case of a 56-year-old female presenting with progressive dysarthria, vertigo, ataxia, and right-sided hemiparesis. MRI revealed a large left parieto-occipital periventricular lesion with mixed solid and cystic components and significant perilesional edema. Given the progressive neurological deterioration, gross total resection of the lesion was performed. Intraoperatively, the tumor exhibited a firm, well-circumscribed morphology. Histopathological examination confirmed the diagnosis of schwannoma, a finding highly unusual in this location.

Conclusion

This case highlights the diagnostic challenges of IS and the importance of surgical resection in achieving favorable neurological outcomes. Given their radiological overlap with aggressive neoplasms, advanced neuroimaging, and careful surgical management are essential for optimizing outcomes. Increased awareness of this rare pathology can aid in early recognition, prevent misdiagnosis, and guide effective neurosurgical strategies.
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来源期刊
Brain & spine
Brain & spine Surgery
CiteScore
1.10
自引率
0.00%
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审稿时长
71 days
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