原发性腹膜后滑膜肉瘤合并妊娠1例报告并文献复习  

Primaryretroperitoneal synovial sarcoma with pregnancy:a case report and literature review

作　　者：查振雷[1] 屈峰[2] 郑金榆[3] 连惠波[2] 甘卫东[2] 张古田[2] 兰厚金[2] 郭宏骞[2] 

机构地区：[1]江苏省江阴市人民医院泌尿外科,江苏江阴214400 [2]南京大学医学院附属鼓楼医院泌尿外科 [3]南京大学医学院附属鼓楼医院病理科

出　　处：《临床泌尿外科杂志》2017年第6期467-471,共5页Journal of Clinical Urology

摘　　要：目的:探讨原发性腹膜后滑膜肉瘤的临床表现、诊断、病理特点及治疗,提高对该疾病的认识。方法:回顾性分析我院1例原发性腹膜后滑膜肉瘤合并妊娠患者的临床资料及随访结果,并结合国内外文献进行分析。结果:本例患者彩超发现腹膜后巨大混杂回声团块时合并妊娠7个月,3个月后剖宫产分娩一健康女婴后予以手术,术前MRI示长T1长T2混杂信号影,大小约16cm×15cm,予行根治性切除术。病理肉眼观示肾脏包膜完整,肾脏及肾上腺被肿瘤包绕,镜下所见为梭形细胞瘤样增生伴黏液变性,免疫组化示WT1、CD56、CD57、CD99、Bc12阳性,分子病理检测到t(18q;v)SYT易位遗传学异常,诊断为原发性腹膜后滑膜肉瘤。术后行TOMO刀放疗及MAID方案化疗,每3个月复查CT,术后随访18个月,尚未见明确复发。结论:原发性腹膜后滑膜肉瘤是非常罕见的、高度恶性的肿瘤,具有恶性程度高,进展迅速、容易复发转移,预后差,生存期短的临床特点。早期诊断及根治性切除并结合放化疗能在一定程度上延长患者生存期。Objective：To raise awareness of primary retroperitoneal synovial sarcoma by investigating the clinical manifestations,pathological characteristics and treatment.Method：We described a retrospective study of a case undergoing diagnostic evaluation and surgery for primary retroperitoneal synovial sarcoma with pregnancy.And a clinical analysis was performed with literature.Result：The patient was a seven-month pregnant woman,and color doppler ultrasound showed mixed echo clumps in retroperitoneal space.MRI showed mixed long T1 and long T2 signal.Then the patient underwent radical resection.By the naked eyes we could see that kidney and adrenal gland were surrounded by tumor,and the kidney capsule was intact.We see microscopically spindle cell tumor hyperplasia with mucous degeneration.Immunohistochemistry showed Bcl2,CD99,WT1,CD56,CD57 were positive.Molecular pathology detected t（18q,v）SYT translocation genetic abnormalities.According to microscopy,immunohistochemical,and molecular neuropathology the pathological diagnosis was primary retroperitoneal synovial sarcoma.TOMO therapy and MAID chemotherapy were performed after hospital discharge.At 18 months postoperatively,she showed no evidence of residual disease or distant metastases.Conclusion：Primary retroperitoneal synovial sarcoma is a very rare,highly malignant sarcoma with quick progression,common recurrence and metastasis,poor prognosis and short survival.Early diagnosis and intraoperative complete resection with postoperative chemoradiotherapy will benefit patients.

关 键 词：原发性腹膜后滑膜肉瘤 妊娠 诊断 病理 治疗 

分 类 号：R738.6[医药卫生—肿瘤]