肺动脉内膜肉瘤临床病理观察  

Pulmonary artery intimal sarcoma: clinical and pathological characteristics

作　　者：蒋依娜[1] 张冠军[1] 杨喆[1] JIANG Yi-na ZHANG Guan-jun YANG Zhe(Department of Pathology, The First Affiliated Hospital of Xi＇ an Jiao Tong University, Xi＇ an, Shanxi 710061, China)

机构地区：[1]西安交通大学第一附属医院病理科,西安710061

出　　处：《诊断病理学杂志》2017年第3期194-197,共4页Chinese Journal of Diagnostic Pathology

摘　　要：目的探讨肺动脉内膜肉瘤(PAIS)临床病理及分子遗传学特点。方法对1例PAIS进行光镜及免疫组化检测,并复习相关文献。结果肿瘤位于肺动脉腔内,由未分化梭形细胞及少量上皮样细胞构成,细胞疏密相间排列,异型性显著,可见黏液样背景和大量胶原纤维间质。免疫组化:vimentin弥漫(+),SMA、desmin和MDM2局灶(+),CK、CD117、ALK、CD34、CD31、myosin、myoglobin、F8和EGFR等均(-),Ki-67阳性指数10%。结论肺动脉内膜肉瘤是发生于肺循环系统的间叶来源恶性肿瘤,很可能起源于动脉内膜下的多能干细胞,多数为未分化肉瘤,可向多种方向分化,预后差。临床表现易误诊为肺动脉栓塞,免疫组化无特异性,根据特定发生部位及组织形态可做出诊断。Purpose To investigate the clinicopathlogical characteristics and molecular genetic alterations of pulmonary artery intimal sarcoma. Methods One case of pulmonary artery intimal sarcoma was studied by immunohistochemical staining. The related literature was reviewed and summarized. Results The tumor was located in the lumen of pulmonary artery. Histopathologically,this case typically displayed a proliferation of spindle cells and in a myxoid background. Most of the spindle cells had significant anaplastic feature. Immunohistochemical stains showed that tumor cells were positive for vimentin. In addition,there were focally positive for SMA,desmin and MDM2. However,the other markers of specific differentiation,such as CK, CD117, ALK, CD34, CD31, myosin, myoglobin, F8 and EGFR were negative.Conclusions Pulmonary artery intimal sarcoma originates from the arterial intima of elastic pulmonary arteries. It is a malignant mesenchymal tumor with poor prognosis. There is no typical clinical symptom and distinctive immunophenotype.A diagnosis can be made by the location and histopathological features.

关 键 词：肺动脉 内膜肉瘤 免疫组化 分子遗传学 

分 类 号：R734.2[医药卫生—肿瘤]