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儿童双侧肾母细胞瘤的治疗:如何提高保肾手术的应用。

Treatment of bilateral Wilms' tumor in children: how to improve the application of nephron-sparing surgery.

机构信息

Department of Urology, Beijing Children's Hospital, National Center for Children's Health, Capital Medical University, No. 56 Nanlishi St, Xicheng District, Beijing, 100045, China.

出版信息

Pediatr Surg Int. 2023 Mar 1;39(1):145. doi: 10.1007/s00383-023-05433-x.

DOI:10.1007/s00383-023-05433-x
PMID:36856873
Abstract

PURPOSE

To summarize the experience of nephron-sparing surgery (NSS) for bilateral Wilms tumors (BWT) in children.

METHODS

This study included children with BWT admitted to our hospital between January 2008 and June 2022. The details of the treatments and outcomes were analyzed.

RESULTS

In all, 70 patients (39 males and 31 females) were enrolled, including 66 patients with synchronous tumors and 4 patients with metachronous tumors. The median age at diagnosis was 13 (3-75) months. Overall, 59 patients received preoperative chemotherapy and 45.8% (54/118) of the 118 sides of WT achieved a partial response (PR). Of the 70 patients, 48 (68.6%) underwent bilateral NSS and 22 (31.4%) underwent unilateral NSS and contralateral total nephrectomy. The proportion of bilateral NSS in the preoperative chemotherapy group was significantly higher than in the non-chemotherapy group (P = 0.031). Additionally, there were 26, 25, 14, and 5 cases of stage I, stage II, stage III, and stage IV, respectively. Among the 70 children, 16 had a recurrence, and 8 died. The 4 years EFS and OS were 67.9% and 89.3%, respectively.

CONCLUSIONS

The long-term survival rates of patients with BWT improved. Hence, preoperative chemotherapy should be administered to enhance the use of NSS in BWT.

摘要

目的

总结儿童双侧 Wilms 瘤(BWT)行保留肾单位手术(NSS)的经验。

方法

本研究纳入 2008 年 1 月至 2022 年 6 月期间在我院就诊的 BWT 患儿,分析其治疗细节及结局。

结果

共纳入 70 例患儿(男 39 例,女 31 例),其中 66 例为同步肿瘤,4 例为异时肿瘤。诊断时的中位年龄为 13(3-75)个月。总体而言,59 例患儿接受了术前化疗,118 个 WT 侧中 45.8%(54/118)达到部分缓解(PR)。70 例患儿中,48 例行双侧 NSS,22 例行单侧 NSS 联合对侧根治性肾切除术。术前化疗组双侧 NSS 的比例明显高于非化疗组(P=0.031)。此外,各期分别为 26、25、14 和 5 例。70 例患儿中,16 例复发,8 例死亡。4 年 EFS 和 OS 分别为 67.9%和 89.3%。

结论

BWT 患儿的长期生存率提高,故应行术前化疗以提高 BWT 中 NSS 的应用。

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本文引用的文献

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Nephron Sparing Surgery in Bilateral Wilms Tumors With Botryoid Growth Pattern.保留肾单位手术治疗双侧具有葡萄簇状生长方式的 Wilms 瘤。
J Pediatr Hematol Oncol. 2022 Apr 1;44(3):e740-e742. doi: 10.1097/MPH.0000000000002337.
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Technical aspects of Nephron-Sparing Surgery (NSS) in children with bilateral centrally located renal tumors.双侧中央型肾肿瘤患儿行保留肾单位手术(NSS)的技术要点。
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Results of the First Prospective Multi-institutional Treatment Study in Children With Bilateral Wilms Tumor (AREN0534): A Report From the Children's Oncology Group.双侧肾母细胞瘤患儿的首个前瞻性多机构治疗研究(AREN0534)结果:来自儿童肿瘤协作组的报告
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Management of bilateral Wilms tumor over three decades: The perspective of a single center.三十多年来双侧肾母细胞瘤的管理:单中心视角
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Bilateral Wilms tumors treated according to the Japan Wilms Tumor Study Group protocol.根据日本肾母细胞瘤研究组方案治疗的双侧肾母细胞瘤。
Pediatr Blood Cancer. 2014 Jul;61(7):1184-9. doi: 10.1002/pbc.24979. Epub 2014 Feb 12.