Kambo Jaspreet S, Richardson Bonnie, Ionescu Diana N, Tucker Tracy, Kraushaar Greg
Can Respir J. 2015 Jan-Feb;22(1):e1-3. doi: 10.1155/2015/231043.
Primary pulmonary synovial sarcoma (PPSS) is a rare malignancy. Its etiology, imaging features and optimal treatment are not well understood. Pulmonary pseudoaneurysms and lymphadenopathy are rare complications of synovial sarcomas. A 40-year-old woman with mild hemoptysis and thoracic back pain underwent a computed tomography scan that revealed multiple pulmonary lesions, paraesophageal lymphadenopathy and incidental bilateral pulmonary emboli. A diagnosis of PPSS was made through the identification of an SS18 translocation by fluorescence in situ hybridization. She was started on adriamycin, ifosfamide and mesna chemotherapy. Over the subsequent two months, she developed three pulmonary artery pseudoaneurysms, ultimately requiring endovascular coiling. Seven months after starting treatment, the patient was asymptomatic. The lesions and lymphadenopathy decreased in size. The present case highlights complications of a rare malignancy and demonstrates positive response to ifosfamide-based chemotherapy in the setting of PPSS.
原发性肺滑膜肉瘤（PPSS）是一种罕见的恶性肿瘤。其病因、影像学特征及最佳治疗方法尚未完全明确。肺假性动脉瘤和淋巴结病是滑膜肉瘤罕见的并发症。一名40岁有轻度咯血和胸背部疼痛的女性接受了计算机断层扫描，结果显示有多个肺部病变、食管旁淋巴结病以及偶然发现的双侧肺栓塞。通过荧光原位杂交检测到SS18易位，从而诊断为PPSS。她开始接受阿霉素、异环磷酰胺和美司钠化疗。在随后的两个月里，她出现了三处肺动脉假性动脉瘤，最终需要进行血管内栓塞治疗。开始治疗七个月后，患者无症状。病变和淋巴结病的大小有所减小。本病例突出了一种罕见恶性肿瘤的并发症，并证明了在PPSS情况下基于异环磷酰胺的化疗有积极反应。
