Intermediate filament proteins and actin isoforms as markers for soft-tissue tumor differentiation and origin. III. Hemangiopericytomas and glomus tumors.

Intermediate filament proteins and actin isoforms as markers for soft-tissue tumor differentiation and origin. III. Hemangiopericytomas and glomus tumors.
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中间丝蛋白和肌动蛋白亚型作为软组织肿瘤分化和起源的标记。

DOI:
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发表时间:
1990
影响因子:
6
通讯作者:
Giulio Gabbiani
Giulio Gabbiani
中科院分区:
医学2区
文献类型:
--
作者:
W. Schurch;O. Skalli;R. Lagacé;T. Seemayer;Giulio Gabbiani

文献摘要

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中间丝蛋白和肌动蛋白亚型的一系列的12个恶性血管外皮细胞瘤和5血管球瘤进行了检查,光学显微镜,透射电子显微镜,二维凝胶电泳(2D-GE),免疫组化,后者使用单克隆抗体或亲和纯化的多克隆抗体结蛋白,波形蛋白,细胞角蛋白,α-平滑肌,α-肌节肌动蛋白。通过光学显微镜,所有的血管外皮细胞瘤披露了一个主要的血管模式,很少storiform,粘液和梭形细胞区,并与不同程度的血管周围纤维化。超微结构显示血管外皮细胞瘤内有平滑肌分化。免疫组化,血管外皮细胞瘤的肿瘤细胞表达波形蛋白作为唯一的中间丝蛋白,缺乏α-平滑肌或α-肌节肌动蛋白。2D-GE仅显示β和γ肌动蛋白,比例典型的成纤维细胞组织。血管球瘤显示血管球细胞内的波形蛋白和α-平滑肌肌动蛋白的免疫组化技术,并披露超微结构不同的平滑肌分化。因此,血管外皮细胞瘤是一种独特的软组织肿瘤,具有统一的形态学、免疫组化和生化特征,最可能与血管球瘤相关,前者代表血管平滑肌细胞的侵袭性和潜在恶性肿瘤,后者代表血管平滑肌细胞的高分化肿瘤。由于恶性血管外皮细胞瘤通过超微结构显示平滑肌分化,但不表达α-平滑肌肌动蛋白,如正常周细胞和血管球细胞,因此建议这些肿瘤代表高度血管化的平滑肌肿瘤,即来自血管平滑肌细胞或其等同物周细胞的低分化平滑肌瘤,其失去了作为分化标志物的α-平滑肌肌动蛋白,其类似于许多常规的低分化平滑肌肉瘤。
Intermediate filament proteins and actin isoforms of a series of 12 malignant hemangiopericytomas and five glomus tumors were examined by light microscopy, transmission electron microscopy, two-dimensional gel electrophoresis (2D-GE), and by immunohistochemistry, the latter using monoclonal or affinity-purified polyclonal antibodies to desmin, vimentin, cytokeratins, alpha-smooth muscle, and alpha-sarcomeric actins. By light microscopy, all hemangiopericytomas disclosed a predominant vascular pattern with scant storiform, myxoid and spindle cell areas, and with variable degrees of perivascular fibrosis. By ultrastructure, smooth muscle differentiation was observed in each hemangiopericytoma. Immunohistochemically, neoplastic cells of hemangiopericytomas expressed vimentin as the sole intermediate filament protein and lacked alpha-smooth muscle or alpha-sarcomeric actins. 2D-GE revealed only beta and gamma actins, in proportions typical for fibroblastic tissues. Glomus tumors revealed vimentin and alpha-smooth muscle actin within glomus cells by immunohistochemical techniques and disclosed ultrastructurally distinct smooth muscle differentiation. Therefore hemangiopericytomas represent a distinct soft-tissue neoplasm with uniform morphologic, immunohistochemical, and biochemical features most likely related to glomus tumors, the former representing an aggressive and potentially malignant neoplasm of vascular smooth muscle cells and the latter a well-differentiated neoplasm of vascular smooth muscle cells. Because malignant hemangiopericytomas disclose smooth muscle differentiation by ultrastructure, but do not express alpha-smooth muscle actin, as normal pericytes and glomus cells, it is suggested that these neoplasms represent highly vascularized smooth muscle neoplasms, ie, poorly differentiated leiomyosarcomas derived from vascular smooth muscle cells or their equivalent, the pericytes, which have lost alpha-smooth muscle actin as a differentiation marker that is similar to many conventional poorly differentiated leiomyosarcomas.