Purpose

To describe the oncologic and surgical management of bilateral Wilms tumor or Wilms tumor arising in a horseshoe kidney with intravenous tumor thrombus to help pediatric surgeons negotiate this rare and difficult anatomic circumstance.

Methods

A single-institution, retrospective medical record review identified 4 cases of bilateral WT and one case of WT arising in a horseshoe kidney with intravenous tumor thrombus between 2009 and 2021. The presentation, imaging, chemotherapy regimen, intraoperative approach, and surgical and oncologic outcomes were reviewed for each of these patients.

Results

All patients received a total of 12 weeks of neoadjuvant chemotherapy. In two patients, a staged approach to the bilateral tumors was undertaken with the first side being operated on after six weeks of therapy and the other side undergoing surgery after an additional six weeks of therapy. Of five patients, four underwent nephron-sparing surgery of all tumors and one underwent unilateral radical nephroureterectomy with contralateral nephron-sparing surgery. Tumor thrombectomy was performed in four of five cases; one patient demonstrated a complete response of the intravenous tumor thrombus to neoadjuvant chemotherapy and did not require thrombectomy. Three patients received adjuvant flank radiotherapy. Three patients developed medically managed stage II or III chronic kidney disease and no patient required renal replacement therapy or kidney transplant to date.

Conclusion

Nephron-sparing surgery is feasible and safe to perform in selected cases of bilateral Wilms tumor with intravascular thrombus by utilizing three-drug neoadjuvant chemotherapy, staged approaches to each kidney when appropriate, and detailed preoperative and/or intraoperative mapping of renal venous anatomy. Successful nephron-sparing surgery with tumor thrombectomy is dependent on a branched renal venous system or the presence of accessory renal veins.

Level of evidence

Level 4.

中文翻译：

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双侧肾母细胞瘤或马蹄肾的血管内血栓管理

目的

描述双侧肾母细胞瘤或马蹄肾中出现肾母细胞瘤并伴有静脉内肿瘤血栓的肿瘤学和外科治疗，以帮助儿科外科医生应对这种罕见且困难的解剖情况。

方法

一项单机构的回顾性医疗记录审查确定了 2009 年至 2021 年间 4 例双侧 WT 和 1 例发生于马蹄肾并伴有静脉内肿瘤血栓的 WT。表现、影像学、化疗方案、术中方法以及手术和肿瘤学结果对这些患者中的每一个进行了审查。

结果

所有患者共接受了 12 周的新辅助化疗。在两名患者中，对双侧肿瘤进行了分期治疗，第一侧在治疗六周后进行手术，另一侧在治疗六周后进行手术。在 5 名患者中，4 名接受了所有肿瘤的保留肾单位手术，1 名接受了单侧根治性肾输尿管切除术和对侧保留肾单位手术。5 例中有 4 例进行了肿瘤血栓切除术；一名患者表现出静脉内肿瘤血栓对新辅助化疗的完全反应，并且不需要血栓切除术。三名患者接受了辅助侧腹放疗。

结论

通过使用三药新辅助化疗、适当时对每个肾脏进行分期入路以及详细的术前和/或术中肾静脉解剖图，在选定的双侧肾母细胞瘤合并血管内血栓病例中进行保留肾单位的手术是可行且安全的。成功的保留肾单位的肿瘤血栓切除术取决于分支的肾静脉系统或副肾静脉的存在。

证据级别

4 级。