原发性肺动脉内膜肉瘤临床病理特征及诊断  

Primary intimal sarcoma of the pulmonary artery: clinicopathologic characteristics and diagnosis

作　　者：张云岗[1] 金木兰[1] 赵宏颖[1] 胡秀梅[1] 

机构地区：[1]首都医科大学附属北京朝阳医院病理科,北京100020

出　　处：《诊断病理学杂志》2014年第5期297-300,共4页Chinese Journal of Diagnostic Pathology

摘　　要：目的探讨肺动脉内膜肉瘤的临床病理特征及诊断。方法分析肺动脉内膜肉瘤的临床表现及影像学特点,观察分析肺动脉内膜肉瘤的病理形态学及免疫组化染色特点,并复习相关文献。结果 2例患者主要症状为咳嗽、胸闷。CT血管显影(CTA)显示右肺动脉主干狭窄、阻塞。组织学形态,肿瘤以梭形细胞为主,部分呈上皮样细胞,不规则排列,细胞间松散,核分裂象丰富,多形性明显,伴有坏死。免疫组化:2例肿瘤细胞vimentin均(+),其中例2 SMA和ALK灶状(+)。其他分化性标记物均(-)。结论肺动脉内膜肉瘤是一种低分化或未分化的间叶性肿瘤,组织学起源不明,预后差。临床上需要与肺动脉血栓栓塞鉴别。Objective To investigate the clinicopathlogical characteristics and the diagnosis of pulmonary artery intimal sarcoma. Methods The characteristics of its clinical symptoms and thoracic imaging, histopathology and immunohistoehemistry were studied in two cases with pulmonary artery intimal sarcoma. Results The two cases presented with cough, chest tightness, and CT angiography （CTA） showed the stenosis and obstruction of the trunk of right pulmonary artery. Histopathologically, the tumors were composed of abundant spindle cells and focal epitheloid cells, which were poorly cohesive. Nuclei were significantly pleomorphie with numerous mitotic figures. The tumor cells were irregularly arranged with focal necrosis. Immunohistochemical stains showed that two cases were both positive for vimentin. In additional, the case 2 was focally positive for SMA and ALK. However, the other markers of specific differentiation were negative in two cases. Conclusion Pulmonary artery intimal sarcoma is a poorly differentiated or undifferentiated menschymal tumor with poor prognosis and of unknown origin. It is necessary to differentiate pulmonary artery intimal sarcoma from chronic pulmonary thromboembolism in a clinical setting.

关 键 词：肺动脉内膜肉瘤 组织病理学 免疫组化 

分 类 号：R734.2[医药卫生—肿瘤]