肺硬化性血管瘤组织中甲状腺转录因子-1的表达及其组织起源的探讨

The expression of thyroid transcription factor-1 in pulmonary sclerosing hemangioma and a further investigation on histogenesis of the tumor

目的 探讨肺硬化性血管瘤 (SH)的组织起源、病理形态特征 ,甲状腺转录因子 1(TTF 1)在SH中的表达及其病理诊断价值。方法 对 17例SH作回顾性研究 ,其中 12例的SH组织标本进行TTF 1、上皮膜抗原 (EMA)、细胞角蛋白 (CK ,pan)、波形蛋白、平滑肌肌动蛋白 (SMA)、胎盘碱性磷酸酶 (PLAP)、嗜铬粒素A(CgA)、突触素、CD34免疫组织化学SP法标记 ;6例作电镜观察。结果 SH组织结构多样 ,主要表现为实性区、乳头区 ,血管瘤样区和硬化区等四种结构 ,瘤细胞主要由表面立方细胞和多角形细胞二种细胞类型组成。所有肿瘤的多角形细胞和表面立方细胞同时表达TTF 1、EMA ,仅 1例见少数多角形细胞CgA、突触素呈弱阳性反应 ,电镜证实这些多角形细胞和胞质中可见少量电子致密颗粒。结论 SH可能起源于多潜能的原始呼吸道上皮细胞 ,它具有向肺泡上皮细胞、Clara细胞分化的能力。TTF

Objective To investigate the histogenesis and pathologic features of pulmonary sclerosing hemangioma (SH) and its expression of thyroid transcription factor 1 (TTF 1). Methods Seventeen cases of pulmonary sclerosing hemangioma were reevaluated and 12 cases were immunostained with a panel of antibodies including TTF 1, EMA, cytokeratin, etc. Six cases were studied by electron microscopy. Results All cases exhibited the classic histologic features with variable proportions of solid, papillary, hemorrhagic or hemangiomatic, and sclerotic pattern. The tumours consisted of solid sheets or aggregates of pale polygonal cells and papillae and clefts lined by cuboidal or columnar cells. Both TTF 1 and EMA expressions were observed in the surface lining cuboidal cells and the pale polygonal cells. In only one case both chromogranin and synaptophysin showed weakly positive in few polygonal cells and a small number of granules with high electron density was found in the polygonal cells by electronic microscopy. Conclusions It is suggested that pulmonary sclerosing hemangioma is an epithelial neoplasm derived from primitive respiratory epithelium capable of differentiating toward pneumocytes or Clara cells. The immunohistochemical markers such as TTF 1, EMA and CK play an important part in diagnosis of SH.