Institute of Pathology, Medical University of Graz, Austria.
Histol Histopathol. 2013 May;28(5):597-603. doi: 10.14670/HH-28.597. Epub 2013 Jan 24.
Elastofibroma dorsi is a benign soft tissue lesion composed of abnormal elastic fibers. Degenerated elastic fibers in skin and liver are associated with clusterin, an apoprotein that shares functional properties with small heat shock proteins. We evaluated the staining pattern and possible role of clusterin in elastofibroma dorsi.
Twenty-one subcutaneous elastofibromas from the scapular region were evaluated with Elastica van Gieson and Orcein stains, immunohistochemically with antibodies to clusterin, smooth muscle actin, S-100, vimentin and CD34 and correlated with clinical data with respect to physical trauma.
Clusterin correlated with the staining pattern of Elastica van Gieson and labelled abnormal broad coarse fibrillar and globular elastic fibers in all elastofibromas. Orcein stains additionally identified fine oxytalan fibers which were not stained by clusterin. Clusterin staining was observed only on the outside of the elastin fibers, while the cores of fibers and globules were unstained. 4/21 elastofibromas showed cellular nodules with a myxoid/collagenous stroma. The round to oval cells showed cytoplasmic staining with vimentin and clusterin; CD34 labelled mostly cell membranes. The cells lacked SMA and S-100 expression. The central areas of the nodules were devoid of elastic fibers, but the periphery contained coarse fibers and globules. 9/ 11 patients, for whom clinical data were available, reported trauma to the scapular region.
Many investigated ED were associated with trauma, which supports a reactive/degenerative etiology of ED. The abnormal large elastic fibers in all ED were enveloped by clusterin. Clusterin deposition may protect elastic fibers from degradation and thus contribute indirectly to the tumor-like presentation of ED.
背阔肌纤维瘤是一种由异常弹性纤维组成的良性软组织病变。皮肤和肝脏中变性的弹性纤维与簇蛋白(clusterin)有关,簇蛋白是一种具有与小热休克蛋白相似功能特性的载脂蛋白。我们评估了簇蛋白在背阔肌纤维瘤中的染色模式和可能的作用。
评估了 21 例肩胛区皮下弹性纤维瘤的 Elastica van Gieson 和 Orcein 染色、簇蛋白、平滑肌肌动蛋白、S-100、波形蛋白和 CD34 的免疫组织化学染色,并结合临床数据评估了与物理创伤的相关性。
簇蛋白与 Elastica van Gieson 的染色模式相关,在所有弹性纤维瘤中标记异常宽而粗的纤维和球形弹性纤维。Orcein 染色还可识别簇蛋白未染色的细 Oxytalan 纤维。簇蛋白染色仅见于弹力纤维的外部,而纤维和球体的核心则未染色。4/21 例弹性纤维瘤显示有黏液样/胶原基质的细胞性小结。圆形至椭圆形细胞显示细胞质波形蛋白和簇蛋白染色;CD34 主要标记细胞膜。这些细胞缺乏 SMA 和 S-100 表达。小结的中心区域无弹性纤维,但周边含有粗纤维和球体。11 例患者中有 9 例(可获得临床数据)报告肩胛区有创伤。
许多研究的 ED 与创伤有关,这支持 ED 的反应性/退行性病因。所有 ED 中的异常大弹性纤维均被簇蛋白包裹。簇蛋白沉积可能保护弹性纤维免受降解,从而间接有助于 ED 的肿瘤样表现。