肺硬化性血管瘤免疫标记及超微结构——提示其细胞的不同来源

Immunohistochemical and ultrastructual study of so-called sclerosing hemangioma of the lung suggests different origins of cells

目的 探讨肺硬化性血管瘤 (so calledsclerosinghemangioma ,S SH )病理形态学特征、免疫标记、组织起源 ,以及表面活性蛋白 B(surfactantprotein B ,SP B)、甲状腺转录因子 1(thyroidtranscriptionfactor 1,TTF 1)等在S SH中表达的意义及其在诊断中的价值。方法 采用透射电镜、免疫组织化学SP法对 30例S SH组织标本的SP B、TTF 1、肥大细胞类胰蛋白酶 (MCT )以及上皮性抗原标记 (CK H、CK L、EMA、CEA)、间皮抗原 (MC)、神经内分泌标记 (NSE、Ch A、突触素、降钙素、ACTH、GH ) ,波形蛋白 (vimentin ,VT)和血管内皮标记的CD34的表达情况进行了观察。结果 S SH由实性区、乳头区、血管瘤样区和硬化区构成 ,相互之间有移行。表面的立方细胞呈单行排列 ,有时可融合成多核巨细胞 ,有短的微绒毛 ,浆内含板层小体 ,免疫组化呈SP B、TTF 1、CK L、EMA、CEA强阳性 ;多角形的间质细胞呈vimentin和TTF 1强阳性 ,而且每例均可见到 2～ 3种神经内分泌标记呈阳性或散在阳性 ,胞浆内可见散在的神经内分泌颗粒和多量的微管。两种细胞CD34及MC均为阴性。实性区内的少数细胞簇呈SP B及EMA阳性。在肿瘤的各部位均可见散在的MCT阳性的肥大细胞。结论 S SH的立方细胞是一种反应性增生的肺泡Ⅱ型细胞 。

Objective To study the morphological characteristics, immunohistochemical stain and histological origin of so called sclerosing hemangioma of the lung (S SH), and to investigate the significance and diagnostic value of expressions of surfactant protein B (SP B), thyroid transcription factor 1 (TTF 1) and other markers in S SH. Methods Using transmission electron microscope and immunohistochemistry methods, the expressions of SP B, TTF 1, mast cell trypsin (MCT), epithelial antigen markers (CK H, CK L, EMA, CEA), mesothelial antigen (MC), neuroendocrine markers (NSE, Ch A, synaptophysin, calcitonin, ACTH, GH), vimentin and CD34 were observed in 30 cases of S SH. Results S SH demonstrated a mixture of four histological patterns: solid, papillary, hemorrhagic and sclerotic pattern, which often showed transitional phenomena. Cuboidal cells on the surface, which contained short microvilli and lamellar bodies in cytoplasm, arranged in one row and sometimes interfused into multinuclear giant cells. Immunohistochemical results showed that these cells demonstrated strongly positive staining to SP B, TTF 1, CK L, EMA and CEA. The other major cell component polygonal stromal cells were strongly positive to vimentin and TTF 1, and positive or weakly positive to 2 or 3 neuroendocrine markers in each case. Sparse neuroendocrine granulae and abundant microtubules were observed in cytoplasm of the cells. Both cuboidal and polygonal cells displayed negative immunohistochemical results to CD34 and MC. Some cell clusters in solid region were positive for SP B and EMA. Mast cells which were positive for MCT existed sparsely in almost full vision field. Conclusion Cuboidal cells of S SH originate from reactive proliferating type Ⅱ pneumocytes and sometimes interfuse into multinuclear giant cells. The polygonal cells in stroma probably originate from multipotential primitive respiratory epithelium and have multiple differentiating ability. The presence of mast cells is also one of histological characteristics of S SH.