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脉络丛乳头状瘤,伴有淀粉样物和弹性物质的基质沉积。

Choroid plexus papilloma with stromal deposition of amyloid and elastic material.

机构信息

Faculty of Medicine, Department of Anatomy, University of La Laguna, Tenerife, Spain.

出版信息

Amyloid. 2010 Jun;17(2):69-74. doi: 10.3109/13506129.2010.483117.

Abstract

Congophilic birefringent amyloid deposits, with immunostaining for transthyretin (TTR) and amyloid P, associated with numerous coarse, enlarged and thick elastic fibres, are reported in the stroma of two choroid plexus papillomas, a finding not previously described in choroid plexus tumours. TTR was expressed as aggregates of 'doughnut-shaped' bodies, in which the TTR-positive peripheral area encircled the elastic fibre (TTR-negative core). Ultrastructurally, the amyloid microfibrils surrounded the elastic fibres and appeared to continue into the microfibrillar mantle of the latter. The stromal TTR-amyloid deposits associated with abundant elastic fibres in tumours that occur in the choroid plexus may be related to the alteration (production/accumulation, insufficient breakdown and/or extracellular matrix modifications) of some of the choroid plexus functions (removal, target and source of polypeptides, including TTR synthesis) and may be of interest for future studies on choroid plexus polypeptide activity and on protein development into elastomeric and amyloidogenic microfibrils.

摘要

嗜刚果红双折射淀粉样物沉积,伴转甲状腺素蛋白(TTR)和淀粉样蛋白 P 的免疫染色,与两个脉络丛乳头状瘤基质中的许多粗大、增大和增厚的弹性纤维相关,这在脉络丛肿瘤中以前没有描述过。TTR 表现为“甜甜圈状”体的聚集,其中 TTR 阳性的外周区域环绕弹性纤维(TTR 阴性的核心)。超微结构上,淀粉样微纤维围绕弹性纤维,并似乎延伸到后者的微纤维帽中。在发生于脉络丛的肿瘤中,与丰富的弹性纤维相关的基质 TTR 淀粉样物沉积可能与某些脉络丛功能的改变(产生/积累、不足的分解和/或细胞外基质修饰)有关(包括 TTR 合成),并可能对未来关于脉络丛多肽活性和蛋白质向弹性和淀粉样微纤维的发育的研究具有意义。

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