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[以囊肿变性为表现的颅骨纤维发育不良病例]

[Case of calvarial fibrous dysplasia presenting with cyst degeneration].

作者信息

Tsutsumi Satoshi, Yasumoto Yukimasa, Ito Masanori

机构信息

Department of Neurosurgery, Juntendo University Urayasu Hospital, Tomioka, Urayasu-city, Chiba, Japan.

出版信息

No Shinkei Geka. 2011 Feb;39(2):163-8.

Abstract

A 49-year-old female developed a painful dysesthesia in the left forehead 10 years after having become aware of a subcutaneous mass accompanied with mild tenderness. Her medical history was unremarkable. Neurological examination showed normal findings with intact facial nerve function. Blood examination was normal. Neuroimaging revealed a cystic bony mass and an adjacent patchy diploic lesion in the left temporal bone. She underwent total tumor resection by frontotemporal craniotomy. Intraoperatively, a bony defect was noted in the lower surface of the patchy lesion communicating with the diploic venous system. A part of the dura mater was invaginated in the bony defect. The patchy lesion was found to communicate with the bony cyst through the diploic structure. Histological appearance of the bony cyst was consistent with the fibrous dysplasia accompanying cyst degeneration. Histological findings of the patchy lesion were identical to those of the bony cyst except for cyst degeneration. We assumed that the diploic venous system and the meningeal vessel might have been associated with the etiology of cyst degeneration in the present case of fibrous dysplasia.

摘要

一名49岁女性在发现左前额皮下肿块并伴有轻度压痛10年后,出现左前额疼痛性感觉异常。她的病史无明显异常。神经学检查显示面神经功能完整,结果正常。血液检查正常。神经影像学检查发现左侧颞骨有一个囊性骨质肿块和一个相邻的斑片状板障病变。她接受了额颞开颅肿瘤全切术。术中发现斑片状病变下表面有一个与板障静脉系统相通的骨质缺损。部分硬脑膜陷入骨质缺损处。发现斑片状病变通过板障结构与骨囊肿相通。骨囊肿的组织学表现与伴有囊肿退变的骨纤维异常增殖症一致。除囊肿退变外,斑片状病变的组织学表现与骨囊肿相同。我们推测,在本病例的骨纤维异常增殖症中,板障静脉系统和脑膜血管可能与囊肿退变的病因有关。

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