Division of Pulmonology, Klinik Schillerhoehe, Center for Pulmonology and Thoracic Surgery, Teaching Hospital of the University of Tuebingen, Solitude Str. 18, 70839, Stuttgart-Gerlingen, Germany,
Lung. 2014 Feb;192(1):211-4. doi: 10.1007/s00408-013-9521-1. Epub 2013 Oct 30.
Pulmonary sarcomas overall are very uncommon and comprise only 0.5 % of all primary lung malignancies. The diagnosis is established only after sarcoma-like primary lung malignancies and a metastatic extrathoracic sarcoma have been excluded. Synovial sarcoma accounts for ~8 % of soft-tissue sarcomas. Synovial sarcoma arising from the pleura has rarely been reported.
We report a case of a 58-year-old woman who complained of right-sided chest pain and shortness of breath. Chest CT scan revealed a large heterogeneous mass, occupying most of the right hemithorax. Histologic diagnosis was supplemented by interphase cytogenetic (FISH) analysis.
Computed tomography guided Tru-cut biopsy was suspicious for a sarcomatous or fibrous malignancy. However, intraoperative frozen-section diagnostics confirmed the diagnosis of a sarcoma. Immunohistochemistry showed that tumor cells expressed epithelial membrane antigen, CD99 and BCL2. Based on immunohistochemistry, the diagnosis of synovial sarcoma was suspected and was confirmed by FISH analysis. The patient was treated with right upper bilobectomy. Due to R1-resection status, postsurgical systemic chemotherapy was administered.
Primary pulmonary synovial sarcoma is a rare primary lung tumor. Due to extensive size of the tumor with pleural and mediastinal invasion only a R1-resection status could be achieved by thoracic surgery.
肺肉瘤总体上非常罕见，仅占所有原发性肺恶性肿瘤的 0.5%。只有排除了肉瘤样原发性肺恶性肿瘤和胸腔外转移性肉瘤后，才能确诊。滑膜肉瘤约占软组织肉瘤的 8%。胸膜起源的滑膜肉瘤很少见。
我们报告了一例 58 岁女性患者，主诉右侧胸痛和呼吸困难。胸部 CT 扫描显示一个大的异质性肿块，占据了右胸腔的大部分。组织学诊断通过间期细胞遗传学（FISH）分析得到补充。
计算机断层扫描引导的 Tru-cut 活检提示为肉瘤样或纤维性恶性肿瘤。然而，术中冷冻切片诊断证实了肉瘤的诊断。免疫组织化学显示肿瘤细胞表达上皮膜抗原、CD99 和 BCL2。基于免疫组织化学，怀疑诊断为滑膜肉瘤，并通过 FISH 分析得到证实。患者接受了右上肺叶双肺叶切除术。由于存在 R1 切除状态，术后给予全身化疗。
原发性肺滑膜肉瘤是一种罕见的原发性肺肿瘤。由于肿瘤体积广泛，伴有胸膜和纵隔侵犯，仅通过胸外科手术即可达到 R1 切除状态。
