Andino Lizmarie, Cagle Philip T, Murer Bruno, Lu Li, Popper Helmut H, Galateau-Salle Francoise, Sienko Anna E, Barrios Roberto, Zander Dani S
Department of Pathology and Laboratory Medicine, University of Texas Health Science Center at Houston Medical School, Houston, Tex, USA.
Arch Pathol Lab Med. 2006 Oct;130(10):1503-9. doi: 10.5858/2006-130-1503-PDTICW.
Desmoid tumors arising in the lung and pleura are extremely rare and can resemble other, more common neoplasms native to these sites. Alterations of the adenomatous polyposis coli/beta-catenin pathway have been detected in sporadic desmoid tumors and have been associated with nuclear accumulation of beta-catenin and overexpression of cyclin D1.
To analyze the expression of beta-catenin and cyclin D1 in desmoid tumors and solitary fibrous tumors (SFTs), and to compare the utilities of these substances for distinguishing between these entities with those of other, more commonly used stains.
Formalin-fixed, paraffin-embedded sections of 4 desmoid tumors (1 pulmonary, 1 pleural, 2 pleural/chest wall), and 5 benign and 6 malignant SFTs of the pleura were immunostained for beta-catenin, cyclin D1, ALK1, CD34, vimentin, desmin, smooth muscle actin, muscle-specific actin, S100, and pancytokeratin. Staining intensity and the percentage of stained tumor cells were assessed semiquantitatively.
Diffuse moderate or strong nuclear staining for beta-catenin was found in all desmoid tumors, 4 of 5 benign SFTs, and 2 of 6 malignant SFTs. All cases except 1 benign SFT showed concurrent cytoplasmic staining. Nuclear and cytoplasmic cyclin D1 staining was increased in all groups. The best distinction between desmoid tumors and SFTs was provided by CD34 (desmoid tumors, 0/4; SFTs, 8/11) and smooth muscle actin (desmoid tumors, 4/4; SFTs, 0/11).
Our findings suggest that alterations in the adenomatous polyposis coli/beta-catenin pathway and cyclin D1 dysregulation may contribute to the pathogenesis of pleuropulmonary desmoid tumors and SFTs. CD34 and smooth muscle actin stains are particularly useful for differentiating between pleuropulmonary desmoid tumors and SFTs.
发生于肺和胸膜的硬纤维瘤极为罕见，可类似于这些部位的其他更常见肿瘤。在散发性硬纤维瘤中已检测到腺瘤性息肉病 coli/β-连环蛋白通路的改变，并与β-连环蛋白的核积聚和细胞周期蛋白 D1 的过表达有关。
分析β-连环蛋白和细胞周期蛋白 D1 在硬纤维瘤和孤立性纤维瘤（SFT）中的表达，并比较这些物质与其他更常用染色剂在区分这些实体方面的效用。
对 4 例硬纤维瘤（1 例肺部、1 例胸膜、2 例胸膜/胸壁）以及 5 例良性和 6 例恶性胸膜 SFT 的福尔马林固定、石蜡包埋切片进行β-连环蛋白、细胞周期蛋白 D1、ALK1、CD34、波形蛋白、结蛋白、平滑肌肌动蛋白、肌肉特异性肌动蛋白、S100 和全细胞角蛋白的免疫染色。对染色强度和染色肿瘤细胞的百分比进行半定量评估。
在所有硬纤维瘤、5 例良性 SFT 中的 4 例以及 6 例恶性 SFT 中的 2 例中发现β-连环蛋白弥漫性中度或强核染色。除 1 例良性 SFT 外，所有病例均同时出现胞质染色。所有组中核和胞质细胞周期蛋白 D1 染色均增加。CD34（硬纤维瘤，0/4；SFT，8/11）和平滑肌肌动蛋白（硬纤维瘤，4/4；SFT，0/11）对硬纤维瘤和 SFT 的区分效果最佳。
我们的研究结果表明，腺瘤性息肉病 coli/β-连环蛋白通路的改变和细胞周期蛋白 D1 失调可能有助于胸膜肺硬纤维瘤和 SFT 的发病机制。CD34 和平滑肌肌动蛋白染色对于区分胸膜肺硬纤维瘤和 SFT 特别有用。
