肺硬化性血管瘤的临床病理特点

Clinical pathological characteristics of sclerosing hemangioma of the lung

目的 探讨肺硬化性血管瘤 (SHL)的临床病理特点及组织来源。方法 对 12例SHL进行临床病理、组化及免疫组化研究。其中 1例作电镜观察。结果 SHL由实性片块区、乳头区、血管瘤样区及硬化区组成。被覆乳头表面、实性区裂隙及血管样腔面的为立方细胞 ;实性区及乳头轴心为形态一致的圆形、多边形胞浆浅染细胞。免疫组化 :立方细胞表达keratin、EMA、CK ;而浅染细胞弥漫表达vimentin及ER、PR ;瘤组织内散在有SY、CgA阳性细胞区。电镜 :肿瘤细胞胞浆内可见致密核心的神经内分泌颗粒。结论 SHL起源于肺间胚层细胞常可伴有神经内分泌细胞增生。ER、PR的高表达可能与女性的高发病率密切相关。

Objective To investigate the clinic pathological characteristics and histogenesis of sclerosing hemangioma of the lung (SHL). Methods Twelve cases of SHL were studied by light microscopy and immunohistochemistry, and one of them was further observed by electron microscopy. Results The tumor showed a mixture of the following four histological patterns, solid, papillary, hemangioma-like and sclerotic. Cuboidal cells covered the papillary projections and cleft-slide spaces in solid areas whereas solid areas and the stalk of papillary projections were composed of uniform, round or polygonal pale cells. Immunohistochemical staining revealed that surface of the cuboidal cells expressed keratin, EMA and CK, and the pale cells expressed vimentin, ER and PR, with some of them showing positive for Syn and CK. Ultrastructurally, there were dense-core neuroendocrine granules in cytoplasm of some tumor cells. Conclusions SHL may originate from the pulmonary mesenchymal cells, often with proliferation of neuroendocrine cells. Expression of ER, PR correlates with predisposition of this tumor to women.