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双侧肾母细胞瘤挽救性手术后的肾功能及预后

Renal function and outcome following salvage surgery for bilateral Wilms' tumor.

作者信息

Kubiak Rainer, Gundeti Mohan, Duffy Patrick G, Ransley Philip G, Wilcox Duncan T

机构信息

Department of Urology, Great Ormond Street Hospital for Children and the Institute of Child Health, London, England, UK.

出版信息

J Pediatr Surg. 2004 Nov;39(11):1667-72. doi: 10.1016/j.jpedsurg.2004.07.009.

Abstract

PURPOSE

Maintaining adequate renal function without compromising cure in children with bilateral Wilms' tumor is surgically demanding and challenging. The aim of this study was to assess renal function and outcome in children treated in one institution for bilateral Wilms' disease by renal salvage procedures.

METHODS

This study is a retrospective review of all patients with histologically proven bilateral nephroblastoma who underwent a renal salvage procedure between November 1973 and June 2002. The median follow-up time was 52 months (range, 8 to 326 months).

RESULTS

Twenty-three patients were included in the study. The median age at diagnosis was 19 months (range, 5 to 65 months). Patients who presented before 1982 (n = 5) were treated surgically first followed by chemotherapy. The remainder (n = 18) received initial chemotherapy before the operation. Of the 46 kidneys, 18 had a nephrectomy. The remaining 28 underwent a renal salvage procedure. At follow-up, 19 patients had good renal function, 2 had satisfactory function, and 2 had renal failure. Seventeen of the 23 children are alive and tumor free at follow-up (74%). Four children died of distant metastases and 2 of renal failure. Local recurrence in the salvaged kidney was detected in 1 patient 12 years after surgery after the commencement of immunosuppression for renal transplantation.

CONCLUSIONS

These results confirm that renal salvage procedures, in combination with chemotherapy, are a safe and effective way of treating children with bilateral Wilms' disease. In addition, renal salvage surgery can maintain satisfactory renal function in the majority of these patients without an increased risk of local recurrence. When transplantation is required, the remaining native kidney should be removed to prevent tumor recurrence.

摘要

目的

在不影响双侧肾母细胞瘤患儿治愈的前提下维持足够的肾功能,手术要求高且具有挑战性。本研究的目的是评估在一家机构接受双侧肾母细胞瘤保肾手术治疗的患儿的肾功能及预后。

方法

本研究是对1973年11月至2002年6月间所有经组织学证实为双侧肾母细胞瘤并接受保肾手术的患者进行的回顾性研究。中位随访时间为52个月(范围8至326个月)。

结果

23例患者纳入研究。诊断时的中位年龄为19个月(范围5至65个月)。1982年前就诊的患者(n = 5)先接受手术治疗,随后进行化疗。其余患者(n = 18)在手术前接受初始化疗。46个肾脏中,18个进行了肾切除术。其余28个接受了保肾手术。随访时,19例患者肾功能良好,2例功能满意,2例出现肾衰竭。23例儿童中有17例在随访时存活且无肿瘤(74%)。4例儿童死于远处转移,2例死于肾衰竭。1例患者在肾移植免疫抑制开始后12年,保肾术后出现局部复发。

结论

这些结果证实,保肾手术联合化疗是治疗双侧肾母细胞瘤患儿的一种安全有效的方法。此外,保肾手术可使大多数此类患者维持满意的肾功能,且局部复发风险未增加。如需进行肾移植,应切除剩余的自体肾以防止肿瘤复发。

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