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突发性前庭神经鞘瘤伴瘤内出血:病例报告及文献复习。

Acute Presentation of Vestibular Schwannoma Secondary to Intratumoral Hemorrhage: A Case Report and Literature Review.

机构信息

Department of Neurosurgery, Tulane Medical Center, New Orleans, Louisiana, USA; Department of Neurosurgery, Ochsner Medical Center, New Orleans, Louisiana, USA.

Department of Neurosurgery, Tulane Medical Center, New Orleans, Louisiana, USA.

出版信息

World Neurosurg. 2019 Sep;129:157-163. doi: 10.1016/j.wneu.2019.05.075. Epub 2019 May 17.

Abstract

BACKGROUND

Vestibular schwannomas (VS) are common slow-growing tumors that typically present with the insidious progression of unilateral hearing loss, tinnitus, vertigo, and gait imbalance. Clinically significant intratumoral acute hemorrhage is exceedingly rare and can present with the acute onset of symptoms, neurologic deterioration, and significant dysfunction of cranial nerves VII and VIII. We discuss a 40-year-old man who developed mild hearing loss and headaches over the course of a month before presenting with a large acutely hemorrhagic vestibular schwannoma. In addition, we review the current literature pertaining to this pathology.

CASE PRESENTATION

A previously healthy 40-year-old man with a 1-month history of mild headaches, dizziness, and left-sided hearing loss, tinnitus, and facial numbness presented with the acute onset of severe headache, vomiting, complete left-sided hearing loss, and left-sided facial weakness. Computed tomography and magnetic resonance imaging revealed a 4.1 × 2.7 cm hemorrhagic mass in the left cerebellopontine angle most consistent with VS. The patient subsequently underwent a retrosigmoid craniotomy and resection of the tumor. Pathology was consistent with hemorrhagic VS. Imaging at 1-year follow-up demonstrated no residual or recurrent disease, and facial motor function had completely recovered.

CONCLUSIONS

Histologically, vascular abnormalities and microhemorrhages are nearly ubiquitous across vestibular schwannomas and may contribute to cystic degeneration and rapid tumor growth. However, clinically significant hemorrhage is rarely encountered and is more commonly associated with more profound neurologic sequelae and cranial nerve VII dysfunction. Surgical resection at the time of presentation should be strongly considered to remove the risk of repeat hemorrhage and further deterioration. Our case represents a typical presentation and clinical course for a patient presenting with this rarely encountered pathology.

摘要

背景

前庭神经鞘瘤(VS)是常见的缓慢生长的肿瘤,通常表现为单侧听力损失、耳鸣、眩晕和步态不平衡的进行性隐匿进展。临床上显著的肿瘤内急性出血极为罕见,可表现为症状的急性发作、神经功能恶化和第七和第八颅神经的显著功能障碍。我们讨论了一名 40 岁男性,他在出现大的急性出血性前庭神经鞘瘤之前,经历了一个月轻度听力下降和头痛。此外,我们回顾了与该病理学相关的当前文献。

病例介绍

一名 40 岁的健康男性,有 1 个月的轻度头痛、头晕和左侧听力下降、耳鸣和面部麻木史,表现为严重头痛、呕吐、左侧完全听力丧失和左侧面部无力的急性发作。计算机断层扫描和磁共振成像显示左侧桥小脑角有一个 4.1×2.7 厘米的出血性肿块,最符合 VS 的诊断。患者随后接受了乙状窦后颅开颅术和肿瘤切除术。病理检查结果符合出血性 VS。1 年随访的影像学检查显示无残留或复发疾病,面部运动功能完全恢复。

结论

组织学上,血管异常和微出血几乎普遍存在于前庭神经鞘瘤中,可能导致囊性变性和肿瘤快速生长。然而,临床上显著的出血很少见,更常见于更严重的神经后遗症和第七颅神经功能障碍。在出现症状时进行手术切除应强烈考虑,以消除重复出血和进一步恶化的风险。我们的病例代表了出现这种罕见病理学的患者的典型表现和临床过程。

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