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颅神经血管母细胞瘤。1例罕见病例报告及文献复习。

Cranial nerve hemangioblastomas. Report of a rare case and review of literature.

作者信息

Roberti Fabio, Jones Robert V, Wright Donald C

机构信息

Department of Neurological Surgery, George Washington University, NW, Washington, DC 20037, USA.

出版信息

Surg Neurol. 2007 Jun;67(6):640-6; discussion 646. doi: 10.1016/j.surneu.2006.08.085. Epub 2007 Feb 15.

Abstract

BACKGROUND

Cranial nerve hemangioblastomas are exceedingly rare lesions. We review available literature and present the first surgical report on a solid hemangioblastoma arising from the trigeminal nerve and involving the Meckel cave that was successfully treated with selective embolization and successive surgical resection.

CASE DESCRIPTION

A 54-year-old woman presented with an 8-month history of facial numbness associated with headache. An MRI revealed a highly vascularized supra- and infratentorial mass involving the right Meckel cave. A preoperative angiogram with selective embolization of tumoral feeders was performed, and the patient elected to undergo surgical treatment. A large solid lesion encasing the trigeminal nerve was resected without complications via a PLPA approach. Hystopathological examination revealed features consistent with the diagnosis of hemangioblastoma. Other stigmata or familiar history of VHL disease were absent.

CONCLUSIONS

Review of the literature confirms that cranial nerve hemangioblastomas are very rare lesions that occur sporadically or in association with VHL disease. Surgical management of such lesions should be directed by clinical and radiological features as well as patient expectations.

摘要

背景

颅神经血管母细胞瘤是极其罕见的病变。我们回顾了现有文献,并首次报告了一例起源于三叉神经并累及Meckel腔的实性血管母细胞瘤,该病例通过选择性栓塞和后续手术切除成功治疗。

病例描述

一名54岁女性,有8个月面部麻木伴头痛病史。MRI显示一个高度血管化的幕上和幕下肿块,累及右侧Meckel腔。术前行血管造影并对肿瘤供血动脉进行选择性栓塞,患者选择接受手术治疗。通过经岩骨后外侧入路(PLPA)切除了包裹三叉神经的大实性病变,无并发症发生。组织病理学检查显示符合血管母细胞瘤的诊断特征。未发现其他VHL病的体征或家族史。

结论

文献回顾证实,颅神经血管母细胞瘤是非常罕见的病变,可散发性发生或与VHL病相关。此类病变的手术治疗应根据临床和影像学特征以及患者期望来指导。

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