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[原发性心脏滑膜肉瘤——一例报告]

[Primary cardiac synovial sarcoma--a case report].

作者信息

Fujioka M, Suehiro S, Shibata T, Kinoshita H, Wakasa K, Haba T

机构信息

Second Department of Surgery, Osaka City University Medical School, Japan.

出版信息

Jpn J Thorac Cardiovasc Surg. 1998 Sep;46(9):923-7. doi: 10.1007/BF03217847.

Abstract

We report a case of cardiac synovial sarcoma, a very rare primary tumor of the heart. The patient was a 29-year-old man with chief complaint of dyspnea. On echocardiography, a tumor 42 mm in diameter occupying the most space of the left atrium was found. The tumor was obstructed the opening of the mitral valve. He was diagnosed with heart failure due to disturbance of the pulmonary venous return and hemodynamic mitral valve stenosis, and underwent surgery. The tumor was a 5-cm sphere originating in the left atrial posterior wall, and was covered with a sheath and solid. The atrial posterior wall was covered with a peel that appeared to be tumor tissue, and this tissue expanded toward the openings of the pulmonary veins bilaterally and the posterior cusp of the mitral valve. The tumor was resected together with the posterior wall. On pathologic examination, the tumor was diagnosed as a synovial sarcoma. Since no other primary focus was found, this tumor was judged to be a primary sarcoma of the heart. The patient was discharged with elimination of symptoms. The sarcoma recurred after 4 months, and the patient underwent a second operation. The recurrent tumor began from the site of resection of the previous operation and expanded up to the inferior pulmonary veins bilaterally. A wide area of the left atrial wall including the tumor was resected, and he was discharged again. The sarcoma recurred again, and he died of progression of heart failure and aggravation of general conditions after 8 months from the first operation.

摘要

我们报告一例心脏滑膜肉瘤,这是一种非常罕见的原发性心脏肿瘤。患者为一名29岁男性,主要症状为呼吸困难。经超声心动图检查,发现一个直径42毫米的肿瘤占据了左心房的大部分空间。该肿瘤阻塞了二尖瓣开口。他因肺静脉回流障碍和血流动力学二尖瓣狭窄被诊断为心力衰竭,并接受了手术。肿瘤为一个起源于左心房后壁的5厘米球形肿物,有包膜且质地坚实。心房后壁覆盖着一层看似肿瘤组织的包膜,该组织向双侧肺静脉开口和二尖瓣后叶扩展。肿瘤连同后壁一并切除。病理检查显示,该肿瘤被诊断为滑膜肉瘤。由于未发现其他原发灶,此肿瘤被判定为原发性心脏肉瘤。患者症状消除后出院。肉瘤在4个月后复发,患者接受了第二次手术。复发性肿瘤从前次手术切除部位开始,双侧向下扩展至肺静脉。包括肿瘤在内的大面积左心房壁被切除,患者再次出院。肉瘤再次复发,自首次手术8个月后,患者因心力衰竭进展和全身状况恶化死亡。

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