腹膜后血管源性平滑肌肉瘤诊疗策略  

Diagnosis and treatment strategies for retroperitoneal vascular leiomyosarcoma

作　　者：刘磊[1] 唐世英[1] 张启鸣 王凯[1] 王国良[1] 张洪宪[1] 侯小飞[1] 肖若陶 马潞林[1] Liu Lei;Tang Shiying;Zhang Qiming;Wang Kai;Wang Guoliang;Zhang Hongxian;Hou Xiaofei;Xiao Ruotao;Ma Lulin(Department of Urology,Peking University Third Hospital)

机构地区：[1]北京大学第三医院泌尿外科,北京100191

出　　处：《重庆医科大学学报》2023年第8期953-957,共5页Journal of Chongqing Medical University

摘　　要：目的:总结临床上少见的腹膜后血管源性平滑肌肉瘤的诊断和治疗策略。方法:自2018年7月至2023年4月,纳入12例腹膜后血管源性平滑肌肉瘤病例,男性3例,女性9例。平均年龄55.2(32.0,69.0)岁。全部为右侧。临床症状包括腰腹部不适、下肢麻木、下肢水肿及腹水。术前误诊为肾上腺皮质癌或副神经节瘤4例,1例未手术行穿刺病理明确。11例手术病例中,后腹腔镜手术4例,其中1例中转为开放;开放手术5例;机器人辅助腹腔镜手术2例。所有病例术中均需进行下腔静脉相关操作,包括夹闭下腔静脉完成右肾静脉修补1例;下腔静脉单纯缝合3例;下腔静脉壁部分切除后单纯修补2例;下腔静脉壁生物补片修补2例;下腔静脉切断再吻合1例;下腔静脉节段性切除2例。结果:肿瘤平均大小10.6(4.2,22.0) cm,平均手术时间268(151,467) min,平均出血量715(20,1 550) mL,术中输血8例,5例同时切除右肾,1例切缘阳性,术后延迟腹膜后出血2例。最终证实,1例平滑肌肉瘤来源于肾上腺中央静脉,2例来源于肾静脉,9例来源于下腔静脉。6例形成下腔静脉内瘤栓。病理显示肿瘤由梭形细胞组成,核分裂象易见。免疫组化所有病例SMA阳性;8例Desmin阳性;Ki-67阳性表达率为20%~80%。平均随访21.6(1.0,54.0)个月。死亡3例;术后复发转移并生存4例;5例无复发,其中3例随访超过2年。结论:腹膜后血管源性平滑肌肉瘤术前容易误诊,术中需处理下腔静脉。手术难度大,应根据情况采用多种手术方式,开放手术首选,后腹腔手术镜选择需谨慎。肿瘤恶性度高,易复发。Objective To investigate the diagnosis and treatment strategies for retroperitoneal vascular leiomyosarcoma which is a rare disease in clinical practice.Methods Related data were collected from 12 patients with retroperitoneal vascular leiomyosarcoma who were treated from July 2018 to April 2023,among whom there were 3 male patients and 9 female patients,with a mean age of 55.2(32.0,69.0)years.The lesion was located in the right side for all patients,and clinical symptoms included abdominal discomfort,lower limb numbness,lower limb,and ascites.Among these patients,4 patients were misdiagnosed with adrenocortical carcinoma or paraganglioma,and 1 patient did not undergo surgery.Among the 11 patients undergoing surgery,4 underwent laparoscopy surgery,among whom 1 was converted to open surgery;5 underwent open surgery;2 underwent robot-assisted laparoscopy.All patients required the operation for the inferior vena cava(IVC)during surgery,including right renal vein repair by clamping the IVC in 1 patient,simple suture of the IVC in 3 patients,partial resection of the IVC wall and simple repair in 2 patients,biological patch repair of the IVC wall in 2 patients,transection and anastomosis of the IVC in 1 patient,and segmental resection of the IVC in 2 patients.Results The 12 patients had a mean tumor size of 10.6(4.2,22.0)cm,a mean time of operation of 268(151,467)minutes,and a mean blood loss of 715(20,1550)mL.8 patients received intraoperative blood transfusion,5 received right nephrectomy,1 had positive surgical margin,and 2 experienced delayed retroperitoneal hemorrhage after surgery.It was confirmed that 1 patient had leiomyosarcoma originating from the adrenal vein,2 had leiomyosarcoma originating from the renal vein,and 9 had leiomyosarcoma originating from the IVC.Tumor thrombus in the IVC was observed in 6 patients.Pathological examination showed that the tumor was composed of spindle cells,with mitotic figures observed.Immunohistochemistry showed positive SMA in all patients and positive Desmin in 8 patients,a

关 键 词：腹膜后 平滑肌肉瘤 血管源性 诊断 治疗 

分 类 号：R615[医药卫生—外科学]