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酷似颅咽管瘤的巨大鞍上Rathke裂囊肿:对一系列外胚层起源的囊性上皮病变的启示

Giant suprasellar Rathke's cleft cyst mimicking craniopharyngioma: implications for a spectrum of cystic epithelial lesions of ectodermal origin.

作者信息

Choudhry Osamah J, Choudhry Asad, Patel Smruti K, Baisre Ada, Eloy Jean Anderson, Liu James K

机构信息

Department of Neurological Surgery, New Jersey Medical School, University of Medicine and Dentistry of New Jersey, Newark, NJ 07101, USA.

出版信息

J Neurol Surg A Cent Eur Neurosurg. 2012 Sep;73(5):324-9. doi: 10.1055/s-0032-1304814. Epub 2012 Jul 30.

Abstract

Cystic epithelial lesions such as Rathke's cleft cysts (RCCs) and craniopharyngiomas may be difficult to distinguish on a clinical, radiographic, and sometimes histopathological basis. We describe a case of a giant 6.5 cm suprasellar cystic lesion that was presumed to be a craniopharyngioma based on the neuroimaging findings. The lesion extended from the anterior skull base and sella turcica to the lateral ventricle and sylvian fissure resulting in obstructive hydrocephalus. Complete surgical removal of the suprasellar lesion was achieved using an extended frontotemporal transbasal skull base approach. Intraoperatively, the cyst wall was thickened and partially calcified, resembling a craniopharyngioma. However, the histopathological examination revealed findings most consistent with a RCC with additional features of extensive squamous metaplasia, metaplastic bone formation, and chronic inflammation. The case raises the issue of whether there is a pathologic continuum of parasellar ectodermal lesions which may account for the overlap of features and transitional states. In this report, we discuss the possible spectrum between RCCs and craniopharyngiomas, and also emphasize the importance of complete resection of the cyst wall in RCCs that exhibit squamous metaplasia, inflammation, or ossification to minimize the probability of recurrence.

摘要

诸如拉克氏裂囊肿(RCCs)和颅咽管瘤等囊性上皮病变在临床、影像学甚至有时在组织病理学基础上可能难以区分。我们描述了一例6.5厘米的巨大鞍上囊性病变病例,根据神经影像学检查结果,该病变被推测为颅咽管瘤。该病变从前颅底和蝶鞍延伸至侧脑室和大脑外侧裂,导致梗阻性脑积水。采用扩大的额颞经基底颅底入路实现了鞍上病变的完整手术切除。术中发现囊肿壁增厚且部分钙化,类似颅咽管瘤。然而,组织病理学检查结果最符合RCC,并伴有广泛鳞状化生、化生骨形成和慢性炎症等额外特征。该病例提出了一个问题,即鞍旁外胚层病变是否存在病理连续谱,这可能解释了特征重叠和过渡状态。在本报告中,我们讨论了RCCs和颅咽管瘤之间可能的范围,同时强调对于表现出鳞状化生、炎症或骨化的RCCs,完整切除囊肿壁对于降低复发概率的重要性。

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