肺硬化性血管瘤18例临床病理分析

目的:探讨肺硬化性血管瘤(SHL)的组织起源及组织成分,增进对其临床病理特征的认识。方法:回顾分析18例SHL临床表现、影像学、病理形态学及免疫组化特征。结果:SHL组织结构主要由实性区、乳头区、出血区和硬化区以不同比例混合而成。89%以上病例SHL的实性区和乳头轴心的圆形间质细胞均对上皮膜抗原(EMA)、甲状腺转录因子(TTF-1)及波形蛋白(VIM)呈阳性反应;50%～67%的病例实性区圆形间质细胞对神经元特异性烯醇化酶(NSE)、突触素(SYN)、雌激素受体(ER)和孕激素受体(PR)呈阳性反应。EMA、TTF-1和表面活性剂脱辅蛋白(PE-10)对乳头表面立方细胞呈阳性反应。结论:SHL组织成分包括胞质浅染的圆形间质细胞和乳头表面立方细胞;肿瘤可能来源于原始未分化的呼吸道上皮。

Clinicopathological analysis of 18 cases of sclerosing hemangioma of the lung

Objective To investigate the histogenesis and composition of sclerosing hemangioma of the lung(SHL) in order to promote the recognition of clinicopathological features of SHL. Methods The radiological, pathological and immunohistochemical features were reviewed in 18 cases of SHL. Results All the patients showed the classical histological features with variable proportions of solid, papillary, haemarrhagic and sclerotic patterns. The positive reactions for EMA, TTF-1 and in vimentin in the surface lining cuboidal cells and in the round stromal cells were revealed over 89% of the cases. NSE, SYN, ER and PR were positive in the solid area in 50%-67% of the cases. Positive reactions for EMA, TTF-1 and PE-10 were observed in the papillary surface cuboidal cells of blood vessels. Conclusions SHL is a very rare lung tumor which is susceptible to women and pathologic diagnosis is often mistakable. SHL consists of pale polygonal cells, papillae and clefts lining cuboidal cells. The tumour may originate from primitive undifferentiated respiratory epithelium.