摘要目的 探讨肺硬化性血管瘤的组织来源.方法 回顾性总结49例肺硬化性血管瘤的临床和病理组织学特征,应用组织芯片及免疫组织化学SP法检测49例硬化性血管瘤中广谱细胞角蛋白(CKpan)、上皮细胞膜抗原(EMA)、波形蛋白、甲状腺转录因子(TTF)-1、napsinA、突触素、嗜铬粒素A(CgA)、CD56、E-cadherin、β-eatenin、CD117、CD68及转化生长因子(TGF) -β1的表达.结果 肿瘤组织中表面立方细胞表达CKpan、EMA、TTF-1、napsin A；多角形细胞表达EMA、TTF-1,不表达CKpan,napsin A的阳性率为16.3％( 8/49).突触素、CgA、CD56两型细胞均呈阴性表达.E-eadherin、β-catenin在49例硬化性血管瘤的表面立方细胞均呈强阳性表达,E-cadherin呈胞膜阳性表达,β-catenin呈胞膜及胞质阳性表达.而多角形细胞E-cadherin不表达(32/49)或弱阳性表达( 17/49),且大部分不表达β-catenin,仅部分呈胞质表达.波形蛋白在所有49例硬化性血管瘤的多角形细胞中均见强阳性表达,其中立方细胞仅见少量细胞阳性表达.CD117及CD68阳性的炎性细胞散在分布于多角形细胞间,TGF-β1阳性表达细胞的分布与CD117及CD68阳性者一致.结论 肺硬化性血管瘤起源于原始呼吸上皮,多角形间质细胞可能来源于立方细胞的转化.TGF-β1可能在硬化性血管瘤上皮细胞间质转化中起着重要的作用.

abstractsObjective To investigate the histogenesis of pulmonary sclerosing hemangioma (PSH).Methods Tissuc microarray and immunohistochemical tcchniquc wcrc used to detect the expression of pancytokeratin,epithelial membrane antigen ( EMA),vimentin,thyroid transcription factor(TTF) -1,napsin A,synaptophysin,chromogranin A,CD56,E-cadherin,β-catenin,CD117,CD68 and transforming growth factor(TGF) -β1 in 49 cases of PSH.Results lmmunohistochemistry revealed that all cuboidal surface cells expressed pan-cytokeratin,EMA,TTF-1 and napsin A.The polygonal cells expressed EMA,TTF-1,napsin A (positive rate 16.3％, 8/49 ), but not pan-cytokeratin. Both types of cells were negative for synaptophysin,chromogranin A and CD56.Strong positive staining for E-cadherin and β-catenin appeared on the membrane of cuboidal cells in all PSH,with cytoplasm staining for β catenin as well.The expression levels of these adhesion molecules decreased in the polygonal cells,with the staining localized to the cytoplasm.E-eadherin staining was not detected or was weak.β-catenin staining was not detected on the cell membrane but partially in the cytoplasm.The polygonal cells stained strongly for vimentin,while only a few cuboidal cells were positive. CD117 and CD68 positive inflammatory cells were scattered between the polygonal cells,which was consistent with the distribution of TGF-β1 positive cells.Conclusions PSH originates from the primitive respiratory epithelium,and polygonal stromal cells may be derived from epithelial-mesenchymal transformation of the cuboidal cells.TGF-β1 may play an important role in the formation of sclerosing hemangioma.