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目的探讨血管内膜肉瘤的临床病理学和免疫学表型特点。方法结合相关文献,对1例内膜肉瘤的临床资料、病理切片和免疫组化标记结果进行分析。结果肿瘤位于左心房内,延伸至右上肺静脉腔内。光镜下肿瘤细胞主要为梭形细胞组成,呈编织状分布,细胞异型性明显,并见散在分布的上皮样细胞和多核瘤巨细胞,核分裂象2~8个/10HPF。免疫组化染色:EGFR()、vimentin()、osteopotin()、SMA(),VEGF、MGMT、CD56、CD99、CD34、AAT及S-100均为灶性阳性,Ki-67>30%;而MyoD1、desmin、CD31、CD117、SA、c-erbB-2、CK、HMB45、melan A、MITF、FⅧRAg、EMA和TTF1蛋白均阴性。结论内膜肉瘤是一种罕见的发生于大血管壁内膜的恶性间叶性肿瘤,免疫表型无特异性,预后差。
Objective To investigate the clinicopathological and immunological characteristics of endometrial sarcoma. Methods The clinical data, pathological sections and immunohistochemical results of 1 case of endometrial sarcoma were analyzed with related literatures. Results The tumor was located in the left atrium and extended to the right upper pulmonary vein. Under light microscopy, the tumor cells mainly consisted of spindle cells, showing a braided distribution with obvious cell atypia. The scattered epithelial cells and multinucleated giant cells were observed. The mitotic figures were 2-8 / 10 HPF. Immunohistochemical staining showed that the positive expression of EGFR, vimentin, osteopotin, SMA, VEGF, MGMT, CD56, CD99, CD34, AAT and S- 30%, while MyoD1, desmin, CD31, CD117, SA, c-erbB-2, CK, HMB45, melan A, MITF, FⅧRAg, EMA and TTF1 were all negative. Conclusions Endometrial sarcoma is a rare malignant mesenchymal tumor that occurs in the intima of the large vessel wall. The immunophenotype is nonspecific and has a poor prognosis.