21例原发性肾脏滑膜肉瘤的临床分析  

Clinical analysis of 21 cases of primary synovial sarcoma of kidney

作　　者：江丽丽 石晨曦[1] 顾冬梅[1] Jiang Lili;Shi Chenxi;Gu Dongmei(Department of Pathology,the First Affiliated Hospital of Soochow University,Suzhou 215000,China)

机构地区：[1]苏州大学附属第一医院病理科,苏州215000

出　　处：《中国医师杂志》2025年第2期265-270,共6页Journal of Chinese Physician

摘　　要：目的探讨原发性肾脏滑膜肉瘤(PRSS)的临床表现、病理学特点、治疗方法及预后,以提高临床对该病的认识及诊疗水平。方法回顾性分析2018-2023年苏州大学附属第一医院及文献报道共21例PRSS患者的临床资料,病理特征,免疫组织化学指标,SYT基因检测,治疗及随访结果,采用Kaplan-Meier法计算年龄与累积生存率的关系。结果全组PRSS患者男女比例为16∶5,左右肾脏比例为7∶14,中位年龄50岁,平均年龄44.5岁,中位肿瘤长径9.7 cm,平均肿瘤长径6.7 cm。57%(12/21)的患者主诉腰痛,48%(10/21)的患者出现肉眼血尿。免疫组织化学检查显示Vimentin(16/16)、BCL-2(18/18)、TLE-1(10/10)均100%阳性,CD99和CD56分别69%(9/13)和85%(6/7)阳性。基因检测92%病例(12/13)见SYT易位信号,二代测序(NGS)检测SS18-SSX1融合2例,SS18-SSX2融合1例,SS18-SSX1+S18-SSX2融合1例,SS18-NEDD4融合1例。2例行术前新辅助化疗,11例术后化疗,2例术后放化疗,5例仅手术。全组患者随访时间3~42个月,4例失访。发病年龄小的患者累积生存时间较长,患者中位生存时间为13个月,平均生存时间为15.4个月。结论PRSS以中年男性多见,右肾易发生,肿物长径常>7 cm,大部分病例可通过病理形态学、免疫表型初诊,荧光原位杂交或者NGS行STY基因检测可提供精确诊断。PRSS预后极差,累积生存率与发病年龄相关,手术切除结合化疗为该病的主要治疗手段。ObjectiveTo investigate the clinical manifestations,pathological features,treatment methods and prognosis of primary renal synovial sarcoma(PRSS)in order to improve the clinical understanding and treatment level of the disease.MethodsThe clinical data,pathological features,immunohistochemical indexes,SYT gene detection,treatment and follow-up results of 21 patients with PRSS reported in the First Affiliated Hospital of Soochow University from 2018 to 2023 were retrospectively analyzed.Kaplan-Meier method was used to calculate the relationship between age and cumulative survival rate.ResultsThe male to female ratio of PRSS patients was 16∶5,the ratio of left and right kidneys was 7∶14,the median age was 50 years old,the mean age was 44.5 years old,the median tumor length diameter was 9.7 cm,and the mean tumor length diameter was 6.7 cm.57%(12/21)reported low back pain and 48%(10/21)reported gross hematuria.Immunohistochemical examination showed 100%positive for Vimentin(16/16),BCL-2(18/18)and TLE-1(10/10),69%(9/13)and 85%(6/7)positive for CD99 and CD56,respectively.Genetic testing found SYT translocation signal in 92%of cases(12/13).Next generation sequencing(NGS)detected SS18-SSX1 fusion in 2 cases and SS18-SSX2 fusion in 1 case.SS18-SSX1+S18-SSX2 fusion was found in 1 case,and one case of SS18-NEDD4 fusion occurred.Preoperative neoadjuvant chemotherapy was performed in 2 cases,postoperative chemotherapy in 11 cases,postoperative chemoradiotherapy in 2 cases,and surgery only in 5 cases.All patients were followed up for 3 to 42 months,and 4 cases were lost to follow-up.Patients with younger onset had a longer cumulative survival time.Patients had a median survival time of 13 months and a mean survival time of 15.4 months.ConclusionsPRSS is more common in middle-aged men,the right kidney is easy to occur,the length of the mass is usually>7 cm,most cases can be initially diagnosed by pathologic morphology,immunophenotype,fluorescence in situ hybridization or NGS STY gene detection can provide accurate diagnosis.

关 键 词：肉瘤 滑膜 肾肿瘤 SYT-SSX 

分 类 号：R73[医药卫生—肿瘤]