儿童双侧肾母细胞瘤手术策略探讨

Surgical strategies for bilateral Wilms tumor in children

目的探讨儿童双侧肾细胞瘤的手术策略及治疗疗效。方法对2009~2020年在上海交通大学医学院附属新华医院小儿外科收治的双侧肾母细胞瘤8例患儿的临床资料、综合治疗及预后进行回顾性分析,其中男5例,女3例,年龄为8.1个月至2.9岁,中位初诊年龄12.2个月。术前化疗方案以VAD方案为主,即长春新碱(0.025 mg/kg)、放线菌素(0.023 mg/kg)及多柔比星(1.200 mg/kg)联用。化疗4~6周后行影像学检查评估肿瘤情况,并采取个体化手术治疗方案。7例经手术治疗的双侧肾母细胞瘤病例均进行定期复查、随访,内容包括影像学检查及肾功能检验。结果8例患儿中初诊时1例存在双肺多发转移,其余病例初诊时不伴有远处转移。4例经临床诊断后予以术前化疗,4例经活检明确诊断后予以术前化疗。其中1例患儿经临床诊断后仅行化疗,未行手术治疗,初诊后22个月因肾衰竭死亡,其余7例均进行手术治疗。4例双侧肾母细胞瘤患儿已完成治疗,其中分期双侧保留肾单位的肾肿瘤切除1例,一侧全切及一侧保留肾单位手术1例,左侧根治性肾切除1例,末次化疗至今分别为11.0年、2.5年及0.4年,无肿瘤进展,无原位及远处转移复发;单侧保留肾单位的左肾肿瘤切除1例,术后4年右侧肿瘤进展,现化疗中。尚有3例仍在术后化疗,其中分期双侧保留肾单位的肾肿瘤切除2例,分期一侧全切及一侧保留肾单位手术1例。在共11个肾母细胞瘤术后病理中(有1例外院肾肿瘤切除,病理不详),混合型最为常见(4/11),其次为胚芽型2例。所有7例接受肿瘤切除术患儿术后均未出现尿液渗漏、肾功能不全等近远期并发症。结论双侧肾母细胞瘤手术应以尽可能保留肾单位为原则,除一侧为较小肿瘤灶可行不影响肾功能的局部剜除的病例,其余病例可行一期双侧保留肾单位的肿瘤切除或一侧根治性肾切除+一侧肿瘤局部剜除术。其他病例尽可能在化疗保护下行分期保留肾单位的肿瘤切除术,以保证一侧肾功能恢复后再行对侧肾肿瘤手术。若一侧预估难以保留肾单位的,可先行对侧保留肾单位的肿瘤切除术,在一侧肾保护下有利于残肾的功能恢复。

Objective Bilateral Wilms tumor is a rare type of Wilms tumor.In most cases,individualized treatment protocols are formulated according to the specific conditions of each case.Here clinical data summary and literature review were combined for exploring the surgical options for bilateral Wilms tumors in children.Methods From 2009 to 2020,retrospective review was performed for clinical data and outcomes of eight children hospitalized with bilateral Wilms tumor at Xinhua Hospital.They accounted for 5%of all Wilms tumors during the last 12 years.There were 5 boys and 3 girls with an age range of 8.1 months to 2.9 years.The most common symptom was abdominal mass(5/8).Only one case had multiple lung metastases upon an initial diagnosis.Results Four cases received preoperative chemotherapy after a clinical diagnosis while the remainders preoperative chemotherapy after a biopsy-confirmed diagnosis.One child received only chemotherapy after a clinical diagnosis and died of renal failure at 22 months after an initial diagnosis.Staged bilateral nephron-sparing tumor resection(n=1),unilateral total resection(n=1),unilateral nephron-sparing tumor resection(n=1)and left nephrectomy(n=1)were performed.All three cases survived without tumor progression,in situ or distant metastasis or recurrence at 11,2.5 and 0.4 years respectively.One case of left nephron-sparing tumor resection was diagnosed with a progression of right Wilm's tumor at 4 years postoperatively.Chemotherapy was ongoing.Three cases received postoperative chemotherapy,including staged bilateral nephron-preserving tumor resection(n=2),unilateral total resection(n=1)and nephron-sparing tumor resection(n=1).Mixed-type Wilms tumors(4/11)predominated.There were no such short/longterm complications as urine leakage or renal insufficiency.Conclusions Bilateral Wilms tumor surgery should be based upon the principle of preserving normal nephron tissue as much as possible.Staged nephron-preserving tumor resection under the protection of chemotherapy is preferred.Restoring unilateral renal function before staged surgery may lower the incidence of renal failure.If preserving normal unilateral nephron tissue is difficult,tumor resection at contralateral side can be performed first and function of residual kidney can be recovered under the protection of another kidney.