儿童肾透明细胞肉瘤合并下腔静脉瘤栓手术1例报告并文献分析

Surgery for renal clear cell sarcoma and inferior vena cava thrombus in children:one case report with a literature review

目的探讨儿童肾透明细胞肉瘤合并下腔静脉瘤栓并瘤栓复发的临床特点、诊治及预后。方法回顾性分析湖南省儿童医院泌尿外科收治的1例肾透明细胞肉瘤并下腔静脉瘤栓患儿临床资料。以“clear cell sarcoma”、“thrombus”和“pediatric”为检索词检索PubMed、Web of Science数据库相关文献;以“透明细胞肉瘤”、“瘤栓”和“儿童”为检索词检索万方数据库及中国知网相关文献;剔除重复病例后进行文献复习,总结儿童肾透明细胞肉瘤的临床特征、手术方式及预后情况。结果本例患儿因“发热3 d、血尿3 d”入院,体查右上腹似可扪及一直径约10 cm肿块,质中,无压痛。腹部CT示肿块不均匀强化,腔静脉内低密度影,腹膜后多处淋巴结肿大。穿刺活检确诊肾透明细胞肉瘤,辅助化疗4个周期后行右肾肿瘤根治、腔静脉瘤栓取出术。2022年3月复查CT发现下腔静脉瘤栓复发,行瘤栓、部分下腔静脉切除术。首次手术时长10 h,术中出血量600 mL,切口一期愈合;病理检查结果:肾透明细胞肉瘤,肿瘤侵范未突破肾被膜,侵犯肾盂壁;肾窦及肾门血管未见肿瘤成分;输尿管切缘及右肾旁淋巴结未见肿瘤细胞,术后放疗6个周期、化疗5个周期;第2次手术时长8 h,出血量300 mL,术后化疗5个周期。病理检查示下腔静脉瘤栓为肿瘤成分。目前已随访至术后6个月,患儿无静脉曲张、下肢水肿等腔静脉梗阻表现,肾功能正常。复查CT未见肿瘤复发及明显肿瘤遗存。共获得9篇符合要求的文献,报道11例儿童肾透明细胞肉瘤合并下腔静脉瘤栓患儿,其中男7例,女4例;平均年龄7岁10个月(1岁11个月至14岁);左肾肿瘤4例,右肾7例;8例瘤栓侵入右心房,9例行肾肿瘤根治切除及腔静脉切开取栓,其中5例在体外循环下取栓。中位随访时间16个月(1.5~36个月),3例出现转移或复发。结论对于肾透明细胞肉瘤合并腔静脉瘤栓患儿,可选择经腔静脉切开取栓;瘤栓复发时,如证实侧支循环畅通,可行瘤栓与部分下腔静脉一同切除。

Objective To report the experience of managing renal clear cell sarcoma with inferior vena cava thrombus and recurrence of tumor thrombus in children.Methods In May 2020,a 15-month-old boy was operated for renal clear cell sarcoma and tumor thrombus in inferior vena cava.The databases of PubMed,Web of Science,Wanfang and CNKI were searched with such keywords as“clear cell sarcoma”and“thrombus”and“pediatric”.Searching time was not limited.Duplicate literatures were excluded and clinical features,surgical approaches and prognoses of renal clear cell sarcoma summarized.Results The child was hospitalized due to fever for 3 days and hematuria for 3 days.On physical examination,a mass with a diameter of around 10 cm appeared in right upper abdomen.There was medium texture and no tenderness.Abdominal computed tomography(CT)indicated uneven enhancement of mass,low-density shadow in vena cava and swelling of multiple retroperitoneal lymph nodes.Renal clear cell sarcoma was confirmed by puncture biopsy.Four cycles of adjuvant chemotherapy were followed by radical right nephrectomy with excision of thrombus.In March 2022,CT re-examination hinted at a recurrence of inferior vena cava thrombus.And resection of tumor thrombus and partial inferior vena cava was performed.Initial operation lasted 10h,intraoperative hemorrhage was 600 mL and the incision by first intention.Pathological examination hinted at renal clear cell sarcoma.Tumor invaded renal pelvis wall without breaching renal capsule;No tumor cell was detected in sinus renalis,peri-renal vessels,ureteral cutting edge or right pararenal lymph node.There were 6 cycles of postoperative radiotherapy and 5 cycles of chemotherapy.A second operation lasted 8 hour with 300 mL hemorrhage.Five cycles of postoperative chemotherapy was administered.Pathological examination indicated that tumor thrombus in inferior vena cava was tumor component.During 6-month follow-ups after reoperation,the child had normal renal function and such non-typical manifestations of vena cava obstruction as varicose veins and edema of lower extremities.CT re-examination revealed no tumor recurrence or obvious tumor residue.A total of 9 eligible literatures were retrieved with 11 children with renal clear cell sarcoma plus inferior vena cava tumor thrombi.There were 7 boys and 4 girls with an average age of 94(23-168)month.The involved side was left(n=4)and right(n=7).Eight tumor thrombi invaded right atrium.Radical nephrectomy and vena cava incision thrombi removal(n=9)and thrombi removal under extracorporeal circulation(n=5)were performed.During a median follow-up period of 16(1.5-36)month,there were 3 cases of metastasis or recurrence.Conclusion Excision is indicated for renal clear cell sarcoma with inferior vena cava thrombus.With a recurrence of tumor thrombus and a patency of collateral circulation of inferior vena cava,tumor thrombus and partial inferior vena cava may be removed together.