背景:涉及收集系统的双侧肾母细胞瘤(BWT)儿童保留肾单位手术(NSS)的文献主要包括病例报告。本研究旨在总结其临床特点,治疗,与肿瘤未涉及收集系统的儿童相比,我们儿科手术中心收治的涉及收集系统的BWT儿童的预后。次要目的是讨论如何在防止肿瘤复发的前提下保留更多的肾实质并防止长期肾衰竭。
方法:回顾了2008年1月至2022年6月在我们的儿科手术中心收治的BWT患者。所有纳入患者根据术中发现的肿瘤与采集系统的关系进行分组。第一组包括肿瘤浸润收集系统的儿童,第二组包括肿瘤生长到收集系统中的儿童,第三组包括肿瘤未累及收集系统的儿童.临床特征,对患者的治疗方法和预后进行分析。
结果:纳入70例患者,包括I组的20例患者,25侧肿瘤浸润收集系统,II组中有10例13侧肿瘤生长到收集系统中,和III组40名患者。Ⅰ组和Ⅱ组患者的年纪和性别差别无统计学意义。总的来说,新辅助化疗后,I组20例患者和II组9例患者出现部分缓解(PR)。在第一组中,25侧肿瘤中有22侧接受了NSS;在II组中,13侧肿瘤中有11侧接受了NSS。平均随访47个月,在第一组中,6/20患者复发,2/20患者死亡;在II组中,3/10患者复发,1/10患者死亡。I组的4年总生存率(OS)差异无统计学意义,II和III(86.36%与85.71%vs.91.40%,P=0.902)。
结论:为了保护肾实质,NSS对于涉及收集系统的BWT儿童是可行的。BWT累及收集体系和不累及收集体系的患者术后远期OS无显著差别。
BACKGROUND: The literature on nephron-sparing surgery (NSS) in children with bilateral Wilms\' tumors (BWT) involving the collection system is mostly comprised of case reports. The present study aimed to summarize the clinical characteristics, treatments, and prognosis of children with BWT involving the collecting system admitted to our pediatric surgery center compared with those whose tumors did not involve the collecting system. A secondary aim was to discuss how to preserve more kidney parenchyma and prevent long-term renal failure under the premise of preventing tumor recurrence.
METHODS: Patients with BWT admitted to our pediatric surgery center between January 2008 and June 2022 were reviewed. All included patients were grouped according to the relationship between the tumor and collecting system according to the intraoperative findings. Group I included children with tumor infiltrating the collecting system, group II included children with tumor growing into the collecting system, and group III included children whose tumor did not involve the collecting system. The clinical features, treatments and prognosis of the patients were analyzed.
RESULTS: Seventy patients were enrolled, including 20 patients with 25 sides of tumors infiltrating the collecting system in group I,10 patients with 13 sides of tumors growing into the collecting system in group II, and 40 patients in group III. There was no significant difference in patients age and gender between group I and group II. In total, 20 patients in group I and 9 patients in group II had partial response (PR) after neoadjuvant chemotherapy. In group I, 22 of 25 sides of tumors underwent NSS; in group II, 11 of 13 sides of tumors underwent NSS. During an average follow-up of 47 months, in group I, 6/20 patients relapsed and 2/20 patients died; in group II, 3/10 patients relapsed and 1/10 patient died. There was no significant difference in 4-year overall survival (OS) rate among groups I, II and III (86.36% vs. 85.71%vs. 91.40%, P = 0.902).
CONCLUSIONS: To preserve renal parenchyma, NSS is feasible for children with BWT involving the collecting system. There was no significant difference in postoperative long-term OS between patients with BWT involving the collecting system and not involving the collecting system.