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诊断挑战:伪装成室管膜瘤的脑室内神经鞘瘤。

Diagnostic challenge: Intraventricular schwannoma masquerading as an ependymoma.

作者信息

Youssef Fahde, Boujguenna Imane, Elhairech Dahmane

机构信息

Neurosurgery Department, HASSAN II Regional Hospital, Guelmim, Morocco.

Faculty of Medicine and Pharmacy of Guelmim, IBN ZOHR University, Guelmim, Morocco.

出版信息

Radiol Case Rep. 2025 Apr 14;20(7):3299-3303. doi: 10.1016/j.radcr.2025.03.075. eCollection 2025 Jul.

Abstract

Intraventricular schwannomas are rare and benign tumors, typically located in peripheral nerves, especially the vestibular nerve. When they occur in the cerebral ventricles, these tumors are particularly unusual, with only 9 documented cases to date. One such case is reported in this article, involving a 10-year-old boy who presented with a seizure, leading to the discovery of an intraventricular mass at the right ventricular junction. After diagnostic evaluation and surgical intervention, the tumor was completely removed, with no postoperative complications. Although surgical management of intraventricular schwannomas is often effective and allows for recovery, their diagnosis remains challenging due to the lack of specific imaging signs. Standard imaging, such as MRI, may confuse these tumors with other brain masses, making early identification difficult. The etiopathogenesis of these tumors remains controversial, and several hypotheses have been proposed to explain their formation, though none have been confirmed. Despite these diagnostic challenges, surgical excision remains the treatment of choice and leads to good outcomes, although further research is needed to better understand the origin of these rare tumors.

摘要

脑室内神经鞘瘤是罕见的良性肿瘤,通常位于周围神经,尤其是前庭神经。当它们发生在脑室时,这些肿瘤尤为罕见,迄今为止仅有9例文献记载。本文报告了这样一例病例,患者为一名10岁男孩,因癫痫发作就诊,结果在右心室交界处发现了一个脑室内肿块。经过诊断评估和手术干预,肿瘤被完全切除,术后无并发症。尽管脑室内神经鞘瘤的手术治疗通常有效且能实现康复,但由于缺乏特异性影像学征象,其诊断仍然具有挑战性。标准影像学检查,如MRI,可能会将这些肿瘤与其他脑肿块混淆,导致早期识别困难。这些肿瘤的病因仍存在争议,尽管尚未得到证实,但已提出了几种假说来解释其形成。尽管存在这些诊断挑战,但手术切除仍然是首选治疗方法,且效果良好,不过仍需要进一步研究以更好地了解这些罕见肿瘤的起源。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8e20/12019006/b6ad8d64664f/gr1.jpg

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