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肩胛骨软骨肉瘤手术切除后经基因分析诊断的放射性诱导的肺滑膜肉瘤

Radiation-induced synovial sarcoma of the lung diagnosed by gene analysis after the surgical resection of chondrosarcoma arising from the scapula.

作者信息

Kubouchi Yasuaki, Taniguchi Yuji, Matsuoka Yuki, Haruki Tomohiro, Fujioka Shinji, Miwa Ken, Nakamura Hiroshige

机构信息

Division of General Thoracic Surgery, Tottori University Hospital, Yonago, Tottori, Japan.

出版信息

Ann Thorac Cardiovasc Surg. 2013;19(2):144-7. doi: 10.5761/atcs.cr.12.01908. Epub 2012 Aug 31.

Abstract

The patient was a 62-year-old male who underwent wide resection and radiotherapy for right scapular chondrosarcoma 12 years ago. An abnormal shadow was detected in the right upper lung field included in the irradiated field on chest X-ray. Since the nodule tended to enlarge, a malignant lung tumor was suspected, and surgery was performed. On histological examination, spindle cells densely proliferated in a bundle pattern. Vimentin, bcl-2 protein, and CD99 were positive, and CD34, cytokeratin, AE1/AE3, and EMA were partially positive on immunohistochemical staining. The SYT-SSX (synaptotagmin- synovial sarcoma X) fusion gene was detected employing RT-PCR, based on which primary synovial sarcoma of the lung was diagnosed. The findings also matched the diagnostic criteria of radiation-induced sarcoma, suggesting radiation-induced primary synovial sarcoma of the lung. Primary synovial sarcoma of the lung is a rare tumor. It is difficult to diagnose based on cellular findings, and immunohistochemical and genetic investigations are essential. Radiation-induced sarcoma may develop through a long-term course, as seen in this patient, for which long-term follow-up after radiotherapy is important.

摘要

该患者为62岁男性,12年前因右肩胛软骨肉瘤接受了广泛切除和放疗。胸部X线检查发现照射野内的右上肺野有异常阴影。由于结节有增大趋势,怀疑为肺恶性肿瘤,遂行手术。组织学检查显示,梭形细胞呈束状密集增殖。免疫组化染色显示波形蛋白、bcl-2蛋白和CD99呈阳性,CD34、细胞角蛋白、AE1/AE3和EMA呈部分阳性。采用逆转录聚合酶链反应(RT-PCR)检测到SYT-SSX(突触结合蛋白-滑膜肉瘤X)融合基因,据此诊断为原发性肺滑膜肉瘤。这些发现也符合放射性肉瘤的诊断标准,提示为放射性诱发的原发性肺滑膜肉瘤。原发性肺滑膜肉瘤是一种罕见的肿瘤。基于细胞表现难以诊断,免疫组化和基因检测至关重要。放射性肉瘤可能经过漫长病程发展,如该患者所示,因此放疗后长期随访很重要。

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