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斜坡骨髓外造血:颅神经麻痹的不常见病因。

Extramedullary hematopoiesis within the clivus: an unusual cause of lower cranial nerve palsy.

机构信息

Department of Neurosurgery, University of Virginia Health System, Charlottesville, VA 22908, USA.

出版信息

J Neurooncol. 2010 Dec;100(3):481-5. doi: 10.1007/s11060-010-0208-7. Epub 2010 May 9.

Abstract

We report one year follow up of a case of extramedullary hematopoiesis within the clivus. The imaging findings, brief clinical course, and endoscopic transphenoidal approach are described. A 29-year-old female with thalassemia developed worsening cranial nerve signs. After imaging studies discovered a large clival mass, she underwent endoscopic transphenoidal biopsy of the lesion. Neural compression from exuberant erythrogenesis within tissue normally quiescent of red blood cell production was found to be the etiology of her neural deficit. Treatment for this condition is generally non-operative unless significant neural compression is present. Radiotherapy and anti-neoplastic agents have been used with success.

摘要

我们报告了一例颅底骨髓外化生的一年随访结果。描述了影像学表现、简要的临床经过和经蝶窦内镜入路。一名 29 岁的女性地中海贫血患者出现进行性颅神经症状。影像学检查发现颅底巨大肿块后,患者接受了经蝶窦内镜下病变活检。发现神经压迫的原因是组织中正常静止的红细胞生成过度活跃。除非存在明显的神经压迫,否则这种情况一般无需手术治疗。放射治疗和抗肿瘤药物已成功应用。

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