Department of Pediatric Surgey.
Department of Cardiothoracic and Vascular Surgery.
J Pediatr Hematol Oncol. 2023 Apr 1;45(3):130-136. doi: 10.1097/MPH.0000000000002510. Epub 2022 Aug 24.
The aim of our study is to present our experience in the management and outcome of Wilms tumor with intracaval thrombus.
All children with Wilms tumor with intracaval thrombus who presented to us from July 2000 to December 2017 were reviewed retrospectively. We evaluated the tumor stage, management, and outcomes in these patients.
Thirty-four patients were included in the study. The median age of presentation was 48 months (11 to 84 mo). Preoperative chemotherapy was given in 32 (94%), with a median duration of 8 weeks. Intracaval thrombus completely resolved in 9 (26%) children after neoadjuvant chemotherapy. Surgical intervention for residual inferior vena cava (IVC) thrombus was performed in 32 patients. The median follow-up was 30 months (5 to 150 mo). At the last follow-up, 24 patients (70%) were alive and disease free. The 5-year overall survival (OS) and event-free survival were 67% (95% confidence interval, 50% to 84%) and 59% (95% confidence interval, 42% to 76%). The OS in children with nonmetastatic disease (94%) was significantly higher than those with metastases (29%; P <0.01). The OS in children with complete resolution of IVC thrombus (100%) was significantly higher than those with persistent thrombus (48%; P =0.025). Analysis of survival outcomes in children with nonmetastatic disease (stage III) revealed no significant difference on comparison with cohort with stage III disease with absence of IVC thrombus. The P -value was 0.224 and 0.53 for 5-year OS and event-free survival, respectively.
The management of Wilms tumor can be complicated by the presence of caval thrombus. Patients with metastasis have a significantly poor outcome. Patients in whom, there is complete resolution of intracaval thrombus on neoadjuvant chemotherapy have a significantly higher OS.
本研究旨在介绍我们在处理合并下腔静脉（IVC）内瘤栓的 Wilms 瘤（WT）患者方面的经验和结果。
回顾性分析 2000 年 7 月至 2017 年 12 月期间我院收治的所有合并 IVC 内瘤栓的 WT 患儿。评估这些患者的肿瘤分期、治疗方法和结局。
本研究共纳入 34 例患儿。中位发病年龄为 48 个月（11 至 84 个月）。32 例（94%）患儿接受了术前化疗，中位化疗持续时间为 8 周。新辅助化疗后，9 例（26%）患儿的 IVC 内瘤栓完全消失。32 例患儿因残留 IVC 瘤栓而行手术干预。中位随访时间为 30 个月（5 至 150 个月）。末次随访时，24 例（70%）患儿存活且无疾病进展。5 年总生存率（OS）和无事件生存率分别为 67%（95%置信区间，50%至 84%）和 59%（95%置信区间，42%至 76%）。无转移患儿的 5 年 OS（94%）显著高于转移患儿（29%；P <0.01）。IVC 内瘤栓完全消失患儿的 5 年 OS（100%）显著高于持续存在患儿（48%；P =0.025）。非转移性疾病（III 期）患儿的生存结果分析显示，与无 IVC 瘤栓的 III 期疾病患儿相比，差异无统计学意义。5 年 OS 和无事件生存率的 P 值分别为 0.224 和 0.53。
WT 可合并 IVC 瘤栓，其治疗较为复杂。存在转移的患儿预后较差。新辅助化疗后 IVC 内瘤栓完全消失的患儿 OS 显著较高。
