肺硬化性血管瘤的组织起源及临床病理特点

Clinicopathlogical characteristics and histogenesis of pulmonary sclerosing haemangioma

目的:探讨肺硬化性血管瘤的临床病理特点、诊断及组织起源。方法:回顾性分析21例肺硬化性血管瘤的临床病理学特点,应用免疫组化检测CKpan、EMA、TTF-1、Napsin-A、Vimentin、ER、PR、CD34、Calretinin、Syn、CgA、CD56及Ki-67在硬化性血管瘤中的表达。结果:21例中女性16例,男性5例;肿瘤主要由实性区、乳头状区、血管瘤样区和硬化区四种结构组成,衬覆的立方上皮和多角形细胞均为瘤细胞,其中2例圆形细胞中见少量脂肪细胞。ER、PR、TTF-1和EMA在两种细胞均表达,Napsin-A和CKpan表达于立方细胞,Vimentin表达于多角形细胞,Syn、CgA、CD56及Ki-67在多角形细胞中部分表达,CD34和Calretinin在两种细胞均不表达。结论:PSH可能起源于原始呼吸道上皮,形态学结合EMA、CKpan、TTF-1、Napsin-A、Vimentin、ER、PR等免疫组化标记可以有效诊断和鉴别诊断PSH,ER、PR的高表达,与女性发病率高密切相关,激素治疗PSH的可能性值得进一步的研究。

Objective：To investigate the clinicopathlogical picture,diagnosis and histogenesis of pulmonary sclerosing haemangioma（PSH）.Methods：The clinicopathological characteristics were retrospectively examined in 21 cases of PSH,and immunohistochemical technique was used to detect the expression of pan-cytokeratin,epithelial membrane antigen（EMA）,thyroid transcription factor（TTF-1）,Napsin-A,vimentin,estrogen（ER）,progestogen（PR）, CD34,calretinin,Syn,CgA,CD56 and Ki-67 in PSH.Results：Of the 21 cases,16 were female and 5,male.The tumors primarily showed four histological manifestations：hemorrhagic,papillary,solid,and sclerotic,with cuboidal surface cells and polygonal stromal cells,and slight adipocytes were seen in the round cells in 2 cases.Immunehistochemistry revealed that all cuboidal surface cells expressed EMA ,TTF-1,Napsin-A,CKpan,ER and PR,whereas round cells were positive with TTF-1,EMA,ER,PR and vimentin,and Syn,CgA,CD56 and Ki-67 were partially expressed in the round cells.CD34 and calreti-nin were negative in all cases.Conclusion：PSH may originate from the primitive respiratory epithelium .Morphological examination combined with immuno-histochemistry detection of CKpan,TTF-1,Napsin-A,vimentin,ER and PR can be helpful in differential diagnosis of this entity.Although higher expression of ER and PR is seen in females of PSH,hormone therapy of PSH remains yet to be determined.