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脑干表皮样囊肿:最新进展

Brainstem epidermoid cyst: An update.

作者信息

Patibandla M R, Yerramneni Vamsi Krishna, Mudumba Vijaya S, Manisha Nukavarapu, Addagada Gokul Chowdary

机构信息

Department of Neurosurgery, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India.

Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India.

出版信息

Asian J Neurosurg. 2016 Jul-Sep;11(3):194-200. doi: 10.4103/1793-5482.145163.

Abstract

The incidence of epidermoid tumors is between 1% and 2% of all intracranial tumors. The usual locations of epidermoid tumor are the parasellar region and cerebellopontine angle, and it is less commonly located in sylvian fissure, suprasellar region, cerebral and cerebellar hemispheres, and lateral and fourth ventricles. Epidermoid cysts located in the posterior fossa usually arise in the lateral subarachnoid cisterns, and those located in the brain stem are rare. These epidermoids contain cheesy and flaky white soft putty like contents. Epidermoid cysts are very slow growing tumors having a similar growth pattern of the epidermal cells of the skin and develop from remnants of epidermal elements during closure of the neural groove and disjunction of the surface ectoderm with neural ectoderm between the third and fifth weeks of embryonic life. We are presenting an interesting case of intrinsic brainstem epidermoid cyst containing milky white liquefied material with flakes in a 5-year-old girl. Diffusion-weighted imaging is definitive for the diagnosis. Ideal treatment of choice is removal of cystic components with complete resection of capsule. Although radical resection will prevent recurrence, in view of very thin firmly adherent capsule to brainstem, it is not always possible to do complete resection of capsule without any neurological deficits.

摘要

表皮样肿瘤的发病率占所有颅内肿瘤的1%至2%。表皮样肿瘤通常位于鞍旁区域和桥小脑角,较少见于外侧裂、鞍上区域、大脑和小脑半球以及侧脑室和第四脑室。位于后颅窝的表皮样囊肿通常起源于外侧蛛网膜下池,位于脑干的则较为罕见。这些表皮样肿瘤含有奶酪样、片状的白色软泥状内容物。表皮样囊肿是生长非常缓慢的肿瘤,其生长模式与皮肤的表皮细胞相似,由胚胎生命第3至5周神经沟闭合及表面外胚层与神经外胚层分离时表皮成分的残余物发展而来。我们报告了一例5岁女孩的脑干内表皮样囊肿病例,囊肿内含有带片状的乳白色液化物质。扩散加权成像对诊断具有决定性意义。理想的治疗选择是切除囊性成分并完整切除包膜。尽管根治性切除可防止复发,但鉴于包膜与脑干紧密粘连且非常薄,完全切除包膜而不造成任何神经功能缺损并不总是可行的。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6cec/4849286/beeb922300cd/AJNS-11-194-g001.jpg

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