所谓肺硬化性血管瘤的临床病理及超微结构观察

Chinicopathological and ultrastructural changes of so-called sclerosing hemangioma in lung

目的 探讨所谓肺硬化性血管瘤 (S SHL)临床病理形态特征和组织来源。方法 对 8例S SHL进行组织学观察及免疫组化检测 ,并对其中 2例进行电镜观察。结果 肿瘤由立方细胞及多角形细胞组成。立方细胞表达Sp B、TTF 1、CK和EMA ,不表达Vim。多角形细胞表达TTF 1和Vim ,部分表达EMA、NSE、Syn、CgA和ACTH ,不表达Sp B和CK。两种细胞均不表达MC、CD34、FⅧRAg、CEA和SMA。超微结构特征是立方细胞部分胞质内可见板层小体 ,多角形细胞部分胞质内可见大量线粒体及粗面内质网 ,少数胞质内见神经分泌颗粒及板层小体。结论 S SHL的立方细胞来源于肺泡Ⅱ型细胞 ,多角形细胞可能起源于肺呼吸道上皮多潜能干细胞。病理诊断依赖于组织学、免疫组化和电镜观察。免疫组化在该病的鉴别诊断中有重要价值。

Purpose To investigate the clinicopathologic features and histogenesis of so-call sclerosing hemangioma of the lung(S-SHL).Methods Eight cases of S-SHL were investigeted by clinicopathological analysis and immunohistochemical stains, in which 2 cases were observed under electron microscope. Results The main histologic appearance was cuboidal cells and pale polygonal cells. Immunohistochemically, the cuoidal cells were positive for Sp-B,TTF-1,CK and EMA, but negetive for vimentin. The pale polygonal cells were positive for TTF-1,but negative for Sp-B and CK . Vimentin, NSE, Syn, CgA, EMA and ACTH showed positive in few pale polygonal cells. The cuboided cells and pale polygonal cells were negative for MC, CD34, FⅧRAg, CEA and SMA. Ultrastructurally, the cytoplasm of the cuboided cells showed annulate lamellae. The pale polygonal cells showed abundant mitochondria, rough endoplasmic reticulum, neurosecretory granules and annulate lamellae. Conclusions The cuboided cells of S-SHL is derved from pulmonary alveolar Ⅱ cell. The pale polygonal cell is derived from primitive respiratory multipotent stem cell. The pathological diagnosis depends on histology, immunohistiochemistry and the observation with electronic microscopy. Immunohistiochemistry plays an important role in the diagnosis.