所谓肺硬化性血管瘤的临床病理及免疫表型研究

Clinicopathological and immunohistochemical characteristics of so-called pulmonary sclerosing hemangioma

目的探讨肺硬化性血管瘤的临床病理特征、免疫表型特点及组织来源。方法应用免疫组化检测26例肺硬化性血管瘤中TTF-1、EMA、Vim、CD34、CD31、CD68、Ⅷ因子、SMA、CK5/6、CK-L、CK-H、CgA、PR及ER的表达,观察病理组织学特征并进行随访。结果肺硬化性血管瘤细胞成分及组织结构复杂,部分病例镜下结构类似类癌、孤立性纤维性肿瘤及非黏液型支气管肺泡癌。肿瘤实质由上皮样细胞及卵圆形细胞构成,组织结构可见实性区、乳头区、血管瘤样区及硬化区。间质可发生脂肪化生及骨化。免疫组化示:两种细胞均表达TTF及EMA,上皮样细胞表达CK、卵圆形细胞表达Vimentin、ER、PR,个别表达Syn。随访未见因肿瘤死亡的病例。结论肺硬化性血管瘤可能起源于呼吸道的原始多潜能干细胞,具有向肺泡Ⅱ型细胞分化的特点。病理诊断中需与类癌、孤立性纤维性肿瘤及非黏液型支气管肺泡癌鉴别。虽有个别病例出现淋巴结转移,但临床预后良好。PR的表达可能与女性的高发病率有关。

Aim To study the clinicopathological and immunohistochemical characteristics and histogenesis of so-called pulmonary sclerosing hemangioma（PSH）.Methods Twenty-six cases of PSH were investigated by light microscopy and immunohistochemistry.Results Four histologic patterns were seen：hemangioma-like,papillary,sclerotic,and solid.Immunohistochemistry staining revealed that the epithelioid cells expressed TTF1,CK,EMA,and the round cells expressed TTF1,vimentin,ER and PR,some Syn.Conclusion PSH may originate from primitive respiratory multipotent stem cell and the differentiation between this tumor and solitary fibrous tumor,bronchioloalveolar carcinoma,or carcinoid tumor of the lung is discussed.Expression of PR correlates with predisposition of this tumor to women.