原发性肺滑膜肉瘤诊断与治疗附1例报告及文献复习

Diagnosis and treatment of primary pulmonary synovial sarcoma： one case report and literature review

目的通过探讨原发性肺滑膜肉瘤的临床症状、影像学表现、病理学及免疫组化，提高对本病的认识。方法报告近期呼吸科确诊1例原发性肺滑膜肉瘤患者，同时对既往报道18例患者进行回顾性研究，分析其临床表现、影像学表现、病理学特征及免疫组化特点。结果临床表现为咳嗽、咳痰或胸痛、胸闷等，影像学表现为肺部肿块影，边缘清楚或呈分叶状，病灶密度不均匀，增强扫描呈不均匀强化，可伴纵隔淋巴结肿大及胸腔积液，病理学检查提示为梭形细胞恶性肿瘤，瘤细胞呈流水状排列，核分裂象易见，免疫组化EMA、Bcl-2、Vimentin等阳性。结论原发性肺滑膜肉瘤临床表现及影像学表现上无特异性，不易与其他肺或胸膜原发性肉瘤相鉴别，需要结合病理及免疫组化结果进行诊断。

Objective To evaluate the clinical manifestations, imaging characteristics, pathology and immunochemistry of primary pulmonary synovial sarcoma and to improve the understanding of the disease. Methods A retrospective study was performed in 18 cases of primary synovial sarcoma combining with one patient with primary synovial sarcoma who was diagnosed recently in Weifang People＇s Hospital, the clinical manifestations, imaging characteristics, pathological features and immunohistochemical characteristics were analyzed. Results Clinical manifestations included cough, expectoration, chest pain, and chest tightness. Chest X-ray and CT scan showed a heterogenous mass in lung with clear margin or lobular shape. Contrast enhanced CT showed heterogeneous enhancement of the mass, intumescent mediastinal lymph node and pleural effusion were seen. Pathological examination showed spindle cell carcinoma with common mitotic count. Immunohistochemistry revealed that EMA, Bcl-2 and Vimentin were positive. Conclusions Primary synovial sarcoma of the lung has no characteristic clinical and imaging manifestations and is difficult to be differentiated from other sarcoma of lung. The diagnosis depends on pathological and immunohistochemistry examination.