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目的探讨血管内膜肉瘤的临床及病理特点。方法对1例肺动脉内膜肉瘤进行光镜、电镜观察和免疫组化检测并复习文献。结果肿瘤位于肺动脉血管腔内,阻塞管腔,并延伸至周围小血管。光镜下肿瘤细胞主要为梭形细胞,散在分布上皮样细胞和多核巨细胞,核分裂象约46/50HPF。电镜观察见细胞内含有丰富的粗面内质网、线粒体,无向特殊细胞分化的特征。免疫组化染色vimentin呈弥漫阳性、α-SMA灶性阳性、HMB-45散在弱阳性,CKpan、CD34、CD31和S-100蛋白等均阴性。结论内膜肉瘤是一种罕见的发生于大血管壁的恶性肿瘤,预后差,免疫表型无特异性,了解该肿瘤发病部位、临床及病理特点有助于做出正确诊断。
Objective To investigate the clinical and pathological features of endometrial sarcoma. Methods One case of pulmonary arterial endometrial sarcoma was examined by light and electron microscopy and immunohistochemically. The literature was reviewed. As a result, the tumor is located in the lumen of the pulmonary artery, obstructing the lumen, and extending to surrounding small blood vessels. Under light microscope, the tumor cells were mainly spindle cells, distributed in epithelial-like cells and multinucleated giant cells, and the mitotic figures were approximately 46/50 HPF. Electron microscopy observations showed that the cells were rich in rough endoplasmic reticulum, mitochondria, and no differentiation to specific cells. Immunohistochemically stained vimentin was diffusely positive, α-SMA focal positive, HMB-45 scattered weakly positive, CKpan, CD34, CD31 and S-100 protein were negative. Conclusion Intimal sarcoma is a rare malignancy that occurs in the large vessel wall. The prognosis is poor, and the immunophenotype is not specific. Understanding the site, clinical and pathological features of the tumor helps to make a correct diagnosis.