Yamaki Minoru, Yonehara Shuji, Noriyuki Toshio
Department of Surgery, Onomichi General Hospital, 1-10-23 Hirahara, Onomichi, Hiroshima, 722-8508, Japan.
Department of Pathology, Onomichi General Hospital, Onomichi, Hiroshima, Japan.
Surg Case Rep. 2017 Dec;3(1):29. doi: 10.1186/s40792-017-0301-7. Epub 2017 Feb 15.
Synovial sarcoma is a malignant neoplasm of soft tissues. It occurs mainly in the extremities and is closely related to tendons, tendon sheaths, and bursal structures. Primary synovial sarcoma of the pleura and lungs is extremely rare.
We present the case of a 62-year-old man with a large synovial sarcoma of the left pleura. He presented with general fatigue and severe dyspnea. Chest computed tomography (CT) revealed a 20-cm tumor in the left thoracic cavity. We first diagnosed the tumor as a sarcomatoid mesothelioma based on CT-guided needle biopsy. We speculated that his severe dyspnea was because of ventilation-perfusion mismatch due to the left pulmonary collapse. Furthermore, we thought that there was a discrepancy between the CT findings and the pathological findings from the biopsy specimen. We performed pleuropneumonectomy through an anterior approach with median sternotomy and 5th-intercostal thoracotomy. The resected specimen contained a 22-cm pleural tumor with parenchymatous hemorrhage. We diagnosed the tumor as monophasic synovial sarcoma based on its morphologic and immunohistochemical features. We suspected there was microscopic residual tumor in the left diaphragm and therefore performed radiation therapy. After radiotherapy, he received adjuvant chemotherapy with ifosfamide and Adriamycin. One year after surgery, the patient is alive with no signs of tumor recurrence.
We report a case of a large synovial sarcoma of the pleura in a patient with severe dyspnea. He was treated with pleuropneumonectomy, radiotherapy, and adjuvant chemotherapy. Although the best treatment for this rare condition has not been defined, we thought that tumor resection and adjuvant therapy were appropriate to control the disease in this case.
滑膜肉瘤是一种软组织恶性肿瘤。它主要发生在四肢，与肌腱、腱鞘和滑囊结构密切相关。胸膜和肺的原发性滑膜肉瘤极为罕见。
我们报告一例62岁男性，患有左胸膜巨大滑膜肉瘤。他表现为全身乏力和严重呼吸困难。胸部计算机断层扫描（CT）显示左胸腔有一个20厘米的肿瘤。基于CT引导下的穿刺活检，我们最初将肿瘤诊断为肉瘤样间皮瘤。我们推测他严重的呼吸困难是由于左肺萎陷导致的通气-灌注不匹配。此外，我们认为CT表现与活检标本的病理结果之间存在差异。我们通过正中胸骨切开术和第五肋间开胸术的前路进行了胸膜肺切除术。切除的标本包含一个22厘米的胸膜肿瘤，伴有实质内出血。基于其形态学和免疫组化特征，我们将肿瘤诊断为单相滑膜肉瘤。我们怀疑左膈存在微小残留肿瘤，因此进行了放射治疗。放疗后，他接受了异环磷酰胺和阿霉素的辅助化疗。手术后一年，患者存活，无肿瘤复发迹象。
我们报告一例患有严重呼吸困难的胸膜巨大滑膜肉瘤患者。他接受了胸膜肺切除术、放疗和辅助化疗。尽管这种罕见疾病的最佳治疗方法尚未确定，但我们认为肿瘤切除和辅助治疗在该病例中适合控制疾病。
