Drozenová J, Povýsil C, Tvrdík D, Babjuk M, Hanus T
Ustav patologie 1, LF UK a VFN, Praha.
Cesk Patol. 2008 Jan;44(1):20-2.
The authors present two cases of primary synovial sarcoma of the kidney. Both patients had a tumor mass in the kidney with vascular invasion of the inferior vena cava and right atrium of the heart in case no. 1. In case no. 2 retroperitoneal lymph node metastasis and multiple metastases to both lungs were observed. Radical nephrectomies were performed in both patients. Histologically, the tumor in case no. 1 was monophasic and in case no. 2 poorly differentiated. Immunohistochemically, vimentin was diffusely positive and a few tumor cells were positive for epithelial membrane antigen. The tumor cells were negative for keratins, S- 100 protein, CD 34, smooth muscle actin, and desmin. In both cases, reverse transcription-polymerase chain reaction using ribonucleic acid extracted from formalin-fixed, paraffin-embedded tissues detected SYT-SSX 1 fusion gene transcripts, which are characteristic molecular findings of synovial sarcoma.
作者报告了两例原发性肾滑膜肉瘤病例。病例1的患者肾脏有肿瘤肿块,伴有下腔静脉和右心房血管侵犯;病例2观察到腹膜后淋巴结转移及双肺多发转移。两名患者均接受了根治性肾切除术。组织学上,病例1的肿瘤为单相型,病例2为低分化型。免疫组化显示,波形蛋白弥漫性阳性,少数肿瘤细胞上皮膜抗原阳性。肿瘤细胞角蛋白、S-100蛋白、CD 34、平滑肌肌动蛋白和结蛋白均为阴性。在这两例病例中,使用从福尔马林固定、石蜡包埋组织中提取的核糖核酸进行逆转录聚合酶链反应,均检测到SYT-SSX 1融合基因转录本,这是滑膜肉瘤的特征性分子表现。