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目的探讨内膜肉瘤(IS)的临床病理学特点、免疫表型、超微结构以及预后等。方法结合相关文献,对1例原发于肺动脉的IS的临床资料、病理切片、免疫组化标记、电镜检测结果进行分析。结果镜下见肿瘤由丰富的梭形细胞组成,呈束状、编织状和不规则状,细胞异型性明显,局部呈上皮样,瘤巨细胞及核分裂象多见。免疫组化标记示肿瘤细胞vimentin和osteopotin弥漫强(+),SMA弥漫(+),CD34及myogenin均灶性区域(+);而MyoD1、myosin、myoglobin、S-100、Des、CK、EMA、CD68以及CD117均(-)。电镜示某些梭形细胞的胞质内可见密体、微肌丝;某些细胞的细胞核周围见大量粗面内质网。结论IS是一种非常罕见的发生于大动脉壁的低分化恶性间叶肿瘤,一般具有纤维母细胞或肌纤维母细胞分化特征,多与染色体基因扩增或获得有关,预后差。
Objective To investigate the clinicopathological features, immunophenotype, ultrastructure and prognosis of endometrial sarcoma (IS). Methods According to the related literatures, the clinical data, pathological sections, immunohistochemical markers and electron microscopic examination results of 1 case of IS in primary pulmonary artery were analyzed. Results Microscopically, the tumor was composed of abundant spindle cells, showing a bundle, braid and irregular shape. The cells showed obvious atypia, epithelial cells, giant cells and mitosis. Immunohistochemical staining showed that vimentin and osteopotin were diffusely (+), SMA diffusely (+), CD34 and myogenin homozygous (+), while MyoD1, myosin, myoglobin, Both CD68 and CD117 (-). Electron microscopy showed some spindle cells in the cytoplasm of the dense body, micro-filament; some cells around the nucleus to see a large number of rough endoplasmic reticulum. Conclusion IS is a very rare poorly differentiated malignant mesenchymal tumor that occurs in the aortic wall. It generally has the characteristics of fibroblasts or myofibroblasts differentiation, mostly associated with the amplification or acquisition of chromosomal genes with poor prognosis.