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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 1及EMA等免疫标记对SH的诊断具有十分重要的价值
Objective To investigate the histological origin and pathological features of pulmonary sclerosing hemangiomas (SH) and the expression of thyroid transcription factor 1 (TTF 1) in SH and its pathological diagnostic value. Methods A retrospective study of 17 cases of SH was performed. Twelve specimens of SH were performed with TTF 1, epithelial membrane antigen (EMA), cytokeratin (CK, pan), vimentin, smooth muscle actin (SMA), and placental base. Phosphatase (PLAP), chromogranin A (CgA), synaptophysin, and CD34 immunohistochemistry SP method markers; 6 cases for electron microscopy. RESULTS: The structure of SH tissues is diverse, mainly manifested in solid area, papillary area, hemangiomas-like area and hardened area. The tumor cells are mainly composed of two types of cells: surface cubic cells and polygonal cells. The tumor cells and surface cuboid cells expressed TTF 1 and EMA at the same time. Only a few cases showed weak positive reaction of CgA and synaptophysin. Electron microscopy confirmed that a small amount of electron dense particles were observed in these polygonal cells and cytoplasm. . Conclusions SH may originate from pluripotent primitive respiratory epithelial cells and it has the ability to differentiate into alveolar epithelial cells and Clara cells. Immune markers such as TTF 1 and EMA have very important value in the diagnosis of SH