王环　池琼

[摘要] 目的 观察肺硬化性血管瘤（pulmonary sclerosing hemangioma，PSH）的临床病理特征，分析免疫组化在PSH中的表达及意义，旨在提高对该病的认识及诊断水平。 方法 收集本院2011年1月～2015年5月PSH患者的临床及病理资料6例进行分析，应用NapsinA、P63、TTF-1、EMA、CK、Vimentin、SP-B、CgA、Syn、CD34、MC共11种抗体进行免疫组化标记。 结果 肿瘤组织主要由乳头状区、实性区、血管瘤样区和硬化区4种组织形态以不同比例混合构成，肿瘤细胞由立方状表面细胞和多角形或圆形间质细胞组成。两种细胞共同表达TTF-1和EMA，立方状表面细胞表达NapsinA、P63、CK和SP-B，多角形或圆形间质细胞表达Vimentin。两种细胞均无MC、CD34及Syn的表达，个别病例多角形或圆形细胞散在表达CgA。 结论 PSH是一种肺内少见的肿瘤，组织学形态多样，易误诊为肺癌。免疫组化检测不仅提示该肿瘤起源于多潜能的原始呼吸道上皮细胞，且在PSH的诊断和鉴别诊断中有重要价值。

[关键词] 肺肿瘤；肺硬化性血管瘤；临床病理；免疫表型；组织起源

[中图分类号] R734.2 [文献标识码] B [文章编号] 1673-9701（2015）35-0106-04

[Abstract] Objective To observe the clinicopathological characteristics of pulmonary sclerosing hemangioma（PSH） and to analyze its expression and significance in immunohistochemistry，in order to improve the understanding and diagnostic level of this diesease. Methods The clinicopathological features of 6 patients with PSH in Whenzhou Center Hospital from January 2011 to May 2015 were analyzed.Using immunohistochemical methods，the expression of NapsinA，P63，TTF-1，EMA，CK，Vimentin，SP-B，CgA，Syn，CD34 and MC were observed. Results PSH exhibited the following histological features：papillary，solid，hemorrhagic，sclerotic，and mixed.The majority of tumors showed a mixed pattern of histology. The tumor was composed of two distinct cellular components，the cuboidal or surface cells and the round or polygonal or stroma cells.Immunohistochemical staining revealed the both kinds of cells expressed TTF-1 and EMA，the cuboidal cells expressed NapsinA， P63， CK and SP-B，the round or polygonal cells expressed Vimentin.Neither of these two types of cells expressed MC，CD34 and Syn.Sporadic expression of CgA was observed in one case. Conclusion PSH is a rare lung tumor，with various histologie appearance，and often misdiagnosed as lung cancer. The immunohistochemical detection not only suggested that the tumor originates from the multiple potential of the primary respiratory epithelium， but also had important value in the diagnosis and differential diagnosis of PSH.

[Key words] Lung neoplasms； Pulmonary sclerosing hemangioma； Clinicopathology； Immunophenotype； Histogenesis

肺硬化性血管瘤（pulmonary sclerosing hemangioma，PSH）是一種少见的肺部良性肿瘤，约占肺原发肿瘤的1%。1956年由Liebow和Hubbel[1]首次报道，该病好发于中年女性，其诱因及发病机制不明。因发病率低，临床及影像学缺乏特异性表现，术前诊断困难，易造成误诊[2]，临床上给患者及家属带来沉重心理负担。又因其组织学表现复杂，在病理诊断尤其是术中冰冻，也易误诊为肺癌[2]，以致手术过度治疗[3]，术后并发症增多，给患者带来本不必要的伤害。因此，提高对该肿瘤的认识及诊断水平尤为重要。本文搜集6例PSH患者的完整病例资料，回顾性分析其临床及病理特征，探讨免疫组化在PSH诊断及鉴别诊断中的应用价值，以便更多的病例得到准确的诊治，现报道如下。……