摘要目的:总结根治性肾切除术联合下腔静脉瘤栓取出术治疗儿童肾母细胞瘤合并下腔静脉瘤栓的临床经验。方法:回顾性分析2014年1月至2019年12月上海交通大学医学院附属新华医院儿普外科收治的17例肾母细胞瘤合并静脉瘤栓患儿的临床资料。其中，男11例，女6例；年龄为(2.9±1.6)岁，范围在8个月至7岁；肿瘤体积为(422.2±245.5)cm 3；肿瘤分期Ⅲ期14例和Ⅳ期3例；瘤栓分级0级2例、Ⅰ级6例、Ⅱ级5例、Ⅲ级2例和Ⅳ级2例。2例未经化疗直接行手术切除，15例活检明确诊断后行新辅助化疗2个疗程，评估肿瘤及瘤栓变化后，再行手术治疗。手术方式包括：经腹手术16例，体外循环下胸腹联合手术1例，其中因瘤栓侵犯下腔静脉壁而行下腔静脉壁部分切除术2例。 结果:所有患儿均顺利完成手术，无围手术期死亡病例。手术时间为190~305 min；术中出血量为(236.3±145.1)ml，范围在100~630 ml。肿瘤组织病理显示均为肾母细胞瘤，其中混合型9例、上皮型4例、局灶间变型2例、间质型1例、胚芽型1例。术后3例发生乳糜漏，为Clavien分级Ⅱ级并发症，予饮食控制后治愈。随访6~55个月，其中13例存活，2例瘤栓复发放弃治疗后死亡，2例术后化疗中严重感染死亡。结论:所有肾母细胞瘤患儿都必须通过影像学检查排除静脉瘤栓的存在与否。根治性肾切除术联合下腔静脉瘤栓取出术治疗是有效、安全的。通过对肿瘤和瘤栓准确分级，采取根治性手术联合化疗、放疗的综合治疗，患儿可以获得较好的预后。

abstractsObjective:To summarize the clinical experiences of radical nephrectomy plus thrombectomy in the treatment of childhood Wilms tumor(WT)with intravascular extension.Methods:From January 2014 to December 2019, clinical data were retrospectively reviewed for 17 WT children with venous extension.There were 11 boys and 6 girls with an average age of(2.9±1.6)(2/3-7)years.The average tumor volume was(422.2±245.5)cm 3.The stage was Ⅲ(n=14)and Ⅳ(n=3). Tumor thrombus was graded as 0(n=2), I(n=6), Ⅱ(n=5), Ⅲ(n=2)and Ⅳ(n=2). Two cases underwent surgery while the remainders received 2 courses of neoadjuvant chemotherapy after a definite preoperative diagnosis by biopsy.Surgical approaches included transabdominal surgery(n=16), thoracic-abdominal surgery plus cardiopulmonary bypass(n=1)and partial resection of inferior vena cava(IVC)wall due to tumor thrombus invading the wall of IVC(n=2). Results:All operations were successfully with no perioperative mortality.Operative duration was(190-305)min and intraoperative volume of blood loss(100-630)(236.3±145.1)ml.Tumor histopathology indicated that all of them belonged to WT and the specific types were mixed(n=9), epithelial(n=4), focal anaplastic(n=2), mesenchymal(n=1)and germ(n=1). Postoperative celiac leakage occurred in 3 cases.And one grade Ⅱ complication of Clavien was cured after a dietary control.During an follow-up period of(6-55)months, there were 13 survivors.Two cases of tumor thrombus recurrence relinquished treatment and another 2 children died of severe infection during postoperative chemotherapy.Conclusions:All children with WT must exclude the presence of venous tumor thrombus.Radical nephrectomy plus thrombectomy is both effective and safe.Through accurate grading of tumors and tumor thrombi and comprehensive treatment of radical surgery plus chemoradiotherapy, WT with venous extension yields a better prognosis.