原发性肺动脉肉瘤的临床病理学研究

Clinicopathologic and Immunohistochemical Study of Primary Pulmonary Artery Sarcoma

目的:探讨原发性肺动脉肉瘤的临床病理特征、免疫表型及预后。方法:对3例(2例女性,1例男性平均年龄41岁)原发性肺动脉肉瘤的临床资料、组织学及免疫组化标记结果进行分析。结果:临床表现为胸闷、气短、进行性加重的呼吸困难,CT及核磁检查可显示肺动脉腔内充盈缺损。病理检查:巨检组织呈肉瘤样、黏液样或胶冻状肿物,紧贴肺动脉内壁生长,并沿血管腔延伸状似血管塑型。镜下肿瘤组织由梭形细胞和黏液样基质构成,均伴有不同程度的坏死。1例诊断为原发性肺动脉内膜肉瘤,2例为平滑肌肉瘤。结论:原发性肺动脉肉瘤是一种起源于肺动脉的罕见恶性肿瘤,其临床及影像学表现均无特异性,确诊依赖于病理组织学及免疫组织化学;临床预后差。

Objective： To study the clinicopathological characteristics, immunohistochemical and prognosis of primary pulmonary artery sarcoma （PAS）. Methods： Three cases of PAS （two female ,one male,average age is 41 years old） were studied by clinical data, histopathologic and immunohistochemical examinations. Results： All patients presented with intermittent chest tightness, shortness of breath and dyspnea. Radiologic studies revealed obstruction of the pulmonary artery. Macroscopically, the PAS appeared as mucoid intra- luminal or nodular sessile mass that spread along the pulmonary artery. Microscopically, the tumor consisted of spindle cells with fascicular and storiform patterns, and was accompanied by necrosis and stromal myxoid change. Immunohistochemically, vimentin, desmin, CD34 were strongly positive and weak reactions of SMA in 1 case. However, vimentin and SMA were strongly positive in the other 2 case. PAS was diagnosed in 1 case, and two were leiomyosarcoma. Conclusions： Primary PAS is a rare tumor of the cardiovascular system. Because ofnonspecific clinical manifestation and radiologic features, PAS is often misdiagnosed for other diseases. The diagnosis of primary PAS should be on the basis of typical morphologic features and immunohistochemical stains. It can not be diagnosed early and often has a poor prognosis.