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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 investigate the clinicopathological features, immunophenotype and prognosis of primary pulmonary sarcoma. Methods: The clinical data, histology and immunohistochemical results of three primary pulmonary sarcomas (two women and one male with a mean age of 41 years) were analyzed. Results: Clinical manifestations of chest tightness, shortness of breath, progressive dyspnea, CT and magnetic resonance imaging can show pulmonary artery filling defect. Pathological examination: giant serous tissue was sarcomatoid, mucinous or jelly-like mass, close to the growth of the pulmonary artery wall, and extending along the vascular lumen-like vascular plasticity. Microscopic tumor tissue by the spindle cells and myxoid matrix, are associated with varying degrees of necrosis. One case was diagnosed as primary pulmonary arterial sarcoma and 2 cases as leiomyosarcoma. Conclusion: Primary pulmonary sarcoma is a rare malignant tumor originated from pulmonary artery. Its clinical and imaging findings are not specific. The diagnosis depends on histopathology and immunohistochemistry. The clinical prognosis is poor.