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目的探讨肺恶性孤立性纤维性肿瘤(MSFT)的临床病理学特征、鉴别诊断及预后。方法结合文献对2例肺MSFT的临床资料、病理学形态及免疫表型进行分析并随访。结果 2例均为男性,年龄分别为49岁和58岁。发生部位均为左肺下叶,均隐匿性起病。肿瘤大小分别为5.6 cm×5.5 cm×3.5 cm及20 cm×18 cm×15 cm。镜检示细胞密集区和细胞稀疏区交替分布,肿瘤细胞间有玻璃样变胶原纤维分隔。密集区肿瘤细胞丰富,呈束状、旋涡状排列,瘤细胞呈短梭形,异型性明显,有肿瘤性坏死和较多核分裂(>4个/10 HPF)。免疫组化示肿瘤细胞CD34、bcl-2、CD99及vimentin(+),Ki-67增殖指数5%~30%;calretinin、S-100、desmin、SMA、CD117、CK、EMA、MC、Dog-1、p53和TTF1均(-)。结论肺MSFT非常罕见,确诊主要依靠病理形态学及免疫组化标记,并应与肺的其他梭形细胞肿瘤进行鉴别。治疗以手术完全切除为主,并注意长期随访。
Objective To investigate the clinicopathological features, differential diagnosis and prognosis of malignant solitary fibrous tumor of lung (MSFT). Methods The clinical data, pathological morphology and immunophenotype of 2 cases of lung MSFT were analyzed and followed up. Results Both cases were male, with the age of 49 and 58 respectively. Occurred sites are lower left lung, are occult onset. The tumor sizes were 5.6 cm × 5.5 cm × 3.5 cm and 20 cm × 18 cm × 15 cm, respectively. Microscopic examination of cell-dense cell and sparse cell alternating distribution of tumor cells between the glass-like collagen fibers separated. Tumor cells in the dense area are abundant, in the form of a bundle, arranged in a swirling pattern. The tumor cells have a short fusiform shape and obvious atypia with tumor necrosis and more nuclear fission (> 4 HPFs). The expressions of CD34, bcl-2, CD99, vimentin (+) and Ki-67 in the tumor cells were detected by immunohistochemistry. The proliferation index of calretinin, S-100, desmin, SMA, CD117, CK, 1, both p53 and TTF1 (-). Conclusions Lung MSFT is very rare. The diagnosis depends mainly on pathomorphology and immunohistochemical markers and should be differentiated from other lung spindle tumors. Surgical treatment of complete removal of the main, and pay attention to long-term follow-up.