Kumar Rajiv, Menon Santosh, Desai Sangeeta B, Pramesh C S, Menon Hari, Jambhekar Nirmala Ajit
Department of Pathology, Tata Memorial Hospital, Parel, Mumbai, India.
Indian J Pathol Microbiol. 2009 Oct-Dec;52(4):520-3. doi: 10.4103/0377-4929.56147.
Primary sarcomas of lung are rare compared to metastatic sarcomas. Herein, we report a rare case of primary pulmonary synovial sarcoma with polypoid endobronchial growth in a 35-year-old lady who presented with cough and dyspnea. A malignant pulmonary tumor was suspected and left pneumonectomy was performed. Grossly, a non-encapsulated polypoidal endobronchial tumor measuring 6 cm in greatest diameter, with a solid, tan-white cut surface was identified. Microscopically, tumor was characterized by a proliferation of oval to spindle-shaped cells arranged in sheets and fascicles. Focal hemangiopericytomatous pattern was noted. Immunohistochemically, tumor cells were positive for vimentin, BCL-2, MIC-2 and calponin and focally positive for pancytokeratin and epithelial membrane antigen. A subsequent molecular analysis performed using reverse transcriptase-polymerase chain reaction with RNA extracted from paraffin-embedded tissue, revealed SYT/SSX1 fusion gene which confirmed the diagnosis of synovial sarcoma. The utility of immunohistochemistry and molecular techniques in diagnosis of such a rare case is stressed and the relevant literature is discussed.
与转移性肉瘤相比，原发性肺肉瘤较为罕见。在此，我们报告一例罕见的原发性肺滑膜肉瘤病例，该病例为一名35岁女性，表现为咳嗽和呼吸困难，肿瘤呈息肉样向支气管腔内生长。怀疑为恶性肺肿瘤，遂行左肺切除术。大体检查发现一个无包膜的息肉样支气管内肿瘤，最大直径为6 cm，切面实性、棕白色。显微镜下，肿瘤的特征是椭圆形至梭形细胞呈片状和束状增生。可见局灶性血管外皮细胞瘤样形态。免疫组化显示，肿瘤细胞波形蛋白、BCL-2、MIC-2和钙调蛋白呈阳性，细胞角蛋白和上皮膜抗原局灶性阳性。随后，使用逆转录聚合酶链反应对石蜡包埋组织提取的RNA进行分子分析，发现了SYT/SSX1融合基因，从而确诊为滑膜肉瘤。强调了免疫组化和分子技术在诊断此类罕见病例中的作用，并讨论了相关文献。
