Department of Pathology, Fudan University Shanghai Cancer Center, Fudan University, Shanghai, China.
Am J Clin Pathol. 2012 Jan;137(1):142-9. doi: 10.1309/AJCP34ZVFLAUTMGL.
Synovial sarcomas arising in unexpected locations may lead to diagnostic challenges. In this report, we describe 3 cases of synovial sarcoma that manifested clinically as primary pericardial lesions. All 3 cases occurred in men in their fourth decade. Fever, cough, chest pain, and chest distress were the most common symptoms. Histologically, 2 of the tumors were spindle cell monophasic, and 1 tumor was biphasic. By immunohistochemical studies, the tumor cells were positive for cytokeratins and epithelial membrane antigen. In addition, the tumor cells displayed focal immunoreactivity for calretinin, cytokeratin 5/6, and HBME-1, resulting in the initial interpretations of malignant mesotheliomas. None of the 3 cases were diagnosed correctly until subsequent molecular cytogenetic assays demonstrated the presence of SYT gene rearrangements. As there are overlapping morphologic features between pericardial synovial sarcoma and mesothelioma, molecular analysis is essential for differential diagnoses.
滑膜肉瘤可发生于意料之外的部位,这可能导致诊断困难。本报告描述了 3 例以原发性心包病变为临床表现的滑膜肉瘤。这 3 例均发生于 40 多岁的男性,最常见的症状是发热、咳嗽、胸痛和胸闷。组织学上,2 例肿瘤为梭形细胞单相型,1 例肿瘤为双相型。免疫组织化学研究显示,肿瘤细胞对细胞角蛋白和上皮膜抗原呈阳性。此外,肿瘤细胞还表现出钙视网膜蛋白、细胞角蛋白 5/6 和 HBME-1 的局灶性免疫反应性,最初的解释为恶性间皮瘤。直到后续的分子细胞遗传学检测显示 SYT 基因重排,这 3 例均未被正确诊断。由于心包滑膜肉瘤和间皮瘤具有重叠的形态学特征,因此分子分析对于鉴别诊断至关重要。