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目的探讨原发性肾脏滑膜肉瘤的临床及病理特点,提高对其诊断、治疗及预后的认识。方法回顾性分析我院2例原发性肾脏滑膜肉瘤的诊治资料并复习国内外文献。结果2例患者均行根治性肾切除术。经镜下检查及免疫组化染色后,病理诊断均考虑为原发性肾脏滑膜肉瘤,进一步应用RT-PCR方法检测出SYT-SSX融合基因mRNA表达,确诊为原发性肾脏滑膜肉瘤。例1术后予以辅助化疗,术后随访14个月未见复发转移。例2术后随访4个月,患者死于肺转移。结论原发性肾脏滑膜肉瘤临床罕见,免疫组化染色对诊断有重要价值,确诊需行分子遗传学检测SYT-SSX融合基因的表达。临床治疗上首选根治性肾切除术辅以化疗,预后极差。
Objective To investigate the clinical and pathological features of primary renal synovial sarcoma and to improve its understanding of diagnosis, treatment and prognosis. Methods Retrospective analysis of 2 cases of primary renal synovial sarcoma in our hospital diagnosis and treatment of literature and review of domestic and foreign literature. Results Both patients underwent radical nephrectomy. After microscopic examination and immunohistochemical staining, the pathological diagnosis was considered as primary renal synovial sarcoma. The mRNA expression of SYT-SSX fusion gene was further detected by RT-PCR and was diagnosed as primary renal synovial sarcoma. Example 1 postoperative adjuvant chemotherapy, no follow-up of 14 months recurrence and metastasis. Example 2 After 4 months of follow-up, patients died of lung metastases. Conclusions Primary renal synovial sarcoma is rare in clinic. Immunohistochemical staining is of great value in the diagnosis. SYS-SSX fusion gene expression is determined by molecular genetic method. The best choice for clinical treatment of radical nephrectomy with chemotherapy, poor prognosis.