原发性肾脏滑膜肉瘤诊疗分析  

Primary synovial sarcoma of the kidney:report of 2 cases and review of literature

作　　者：张墨[1] 宋永胜[1] 单立平[1] 王晓彬[1] 

机构地区：[1]中国医科大学附属盛京医院第二泌尿外科,沈阳110004

出　　处：《中国肿瘤临床与康复》2010年第2期152-155,共4页Chinese Journal of Clinical Oncology and Rehabilitation

摘　　要：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 and to improve the recognition of diagnosis,treatment and prognosis of primary renal synovial sarcoma.Methods Two cases of primary renal synovial sarcoma were analyzed retrospectively and the literature was reviewed.Results Both cases underwent radical nephrectomy.The diagnosis was considered to be primary renal synovial sarcoma after pathological examination in both cases.The SYT-SSX fusion transcripts were detected by a reverse transcript-polymerase chain reaction（RT-PCR）,therefore the diagnosis of primary renal synovial sarcoma was finally confirmed.Case 1 received adjuvant chemotherapy.There was no recurrence and metastasis during 14 months follow-up period.Case 2 died of pulmonary metastasis 4 months after surgery.Conclusion Primary renal synovial sarcoma is an extremely rare entity.Immunohistochemical staining is important to diagnosis.SYT-SSX mRNA detection by molecular genetics is imperative for final diagnosis.Surgery and adjuvant chemotherapy are the preferred treatment option.The prognosis is poor.

关 键 词：肾肿瘤 免疫组织化学 SYT-SSX融合基因 

分 类 号：R737.11[医药卫生—肿瘤] R730.5[医药卫生—临床医学]