原发性肺动脉内膜肉瘤的临床病理观察  

Primary pulmonary artery intimal sarcoma: a case report and review of literature

作　　者：樊祥山[1] 王益华[1] 乐美照[2] 吕翔[1] 李桂梅[2] 高凤娟[3] 孟奎[1] 周正扬[4] 贾支俊[5] 

机构地区：[1]南京大学医学院附属鼓楼医院病理科,南京210008 [2]解放军第81医院病理科,南京210002 [3]南京大学医学院附属鼓楼医院急诊中心,南京210008 [4]南京大学医学院附属鼓楼医院放射科,南京210008 [5]南京大学医学院附属鼓楼医院核医学科,南京210008

出　　处：《诊断病理学杂志》2007年第2期105-109,共5页Chinese Journal of Diagnostic Pathology

摘　　要：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 study the clinicopathological, , ultrastructural features and prognosis of intimal sarcoma （IS）. Methods The clinical data, histopathological changes, immunophenotype, ultrastructural features and prognosis of a case of pulmonary aortic IS were studied and relative literature was reviewed. Results Microscopically, the tumor was predominantly composed of abundant spindle cells with obvious dysplasia, which revealed bunchy, weaving and irregular patterns. The tumor cells had varying degrees of dysplasia, mitotic activity, necrosis and nuclear polymorphism. Myxoid areas, hemorrhage and epithehoid appearance of tumor cells were seen stains of vimentin and esteopotin were strong positive and SMA positive in tumor cells. CD34 and myogenin were positive only in local areas. However, MyoD1, myosin, myoglobin, S100, desmin, CK, EMA, CD68 and CD117 were negative. Ultrastructurally, microfdments, dense bodies or much rough endoplasmic reticulum were seen in cytoplasm or nearby karyon. Conclusion IS, usually arising in large arerial blood vessels, is a very rare poorly differentiated mesenchymal tumor with fibroblastic and myofibroblastic differentiation. It can not be diagnosed early and often has a poor prognosis.

关 键 词：肺动脉 内膜肉瘤 临床病理学 

分 类 号：R732.21[医药卫生—肿瘤]