孤立性纤维性肿瘤临床病理学观察

Clinical and pathological characteristics of solitary fibrous tumors

目的探讨孤立性纤维性肿瘤(solitary fibrous tumor,SFT)的临床、病理组织学、免疫组化特征,提高对该病的认识。方法对7例SFT病例进行大体观察、病理组织学及免疫组化En V ision二步法标记,观察V im、CD34、CD99、bc l-2 Des、SMA、HMBE-1、CK、S-100蛋白在SFT组织中的表达。结果7例SFT分别发生于胸腔、腹腔、纵隔、眼眶及胸壁软组织,临床表现为肿块压迫所致的症状,肿块1.5 cm×1.5 cm×1 cm^20 cm×20 cm×18 cm,光镜下肿瘤细胞由长梭形细胞构成束状、编织状排列,间质内有多少不一的胶原纤维,无特殊的组织构型及形态的多样性为其组织学特点。免疫表型:CD34、V im、CD99均为弥漫强阳性,bc l-2 5例阳性,Des、SMA、HMBE-1、CK、S-100蛋白阴性。结论SFT是一组形态多样、无特殊组织构型及有多少不一胶原纤维的梭形细胞肿瘤,其特殊的免疫表型,有助于对该病的诊断,但SFT的确诊必须结合组织形态和免疫组化共同完成。SFT的生物学行为取决于其组织学形态和肿块的大小及生长方式,完全切除肿块者预后较好。

Purpose To assess whether new markers could help to make diagnosis of the solitary fibrous tumor （SFT）, and to study its clinical, histopathologic and immunohistochemical characteristics. Methods Tumor tissues from seven cases of SFT were analyzed in this study. Vim, CD34, CD99, bcl-2, Des, SMA, HMBE-1, CK and S-100 protein antibodies were used to detect their expression in the tumors by an immunohistochemical EnVision technique. Results Seven cases of SFT were found in thoracic cavity, abdomen, mediastinum, fossa orbitalis and soft tissue in the chest wall respectively. Majority of the patients demonstrated clinical symptoms due to compression from a large tumor. The size of tumor was ranged from 1.5 cm × 1.5 cm × 1.0 cm to 20 cm × 20 cm × 18 cm. Spindle-shaped cells that densely arranged in fascicule and storiform were observed under microscope. Furthermore, a pattern of these tumor cells showing interstitial collagen deposition and a patternless pattern were also found. Immunohistochemically, over-expression of Vim, CD34 and CD99 were observed in all these patients. But only 5 tumors showed bcl-2 positivity. Des, SMA, HMBE-1, CK and S-100 protein were negative. Conclusions SFT is a neoplasm in which variable spindle cells, patternless pattern and collagen deposition can be observed, but it is hard to get a correct diagnosis of the SFT without immunohistochemical analysis. In this study, the high expression of the Vim, CD34 and CD99 in all tumor samples suggests that these markers can be used to identify SFT. However the outcome in patients with SFT depends on factors including tumor size and biological behavior. Surgery is still the first choice for the treatment. If tumor could be removed completely, the patients may have a good prognosis.