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涉及肝静脉汇合处和下腔静脉的小儿肝脏肿瘤的手术策略

Surgical Strategy for Pediatric Liver Tumors Involving the Hepatic Venous Confluence and the Inferior Vena Cava.

作者信息

Fuchs Juri, Rabaux-Eygasier Lucas, Hery Geraldine, Fouquet Virginie, Guerin Florent, Franchi-Abella Stephanie, Branchereau Sophie

机构信息

Department of General, Visceral, Pediatric and Transplantation Surgery, Heidelberg University Hospital, Heidelberg, Germany.

Department of Pediatric Surgery, Hôpital Kremlin-Bicêtre, APHP, University of Paris-Saclay, Paris, France.

出版信息

Ann Surg Oncol. 2025 Mar 26. doi: 10.1245/s10434-025-17245-5.

Abstract

BACKGROUND

Pediatric liver tumors presenting as centrally located masses with contact to or even invasion of all three hepatic veins (HVs) and the inferior vena cava (IVC) present significant surgical challenges. While liver transplantation may be indicated in truly unresectable tumors, extended liver resection with vascular reconstruction can be an organ-preserving alternative.

OBJECTIVE

This study aimed to present a reference center's strategy for children with liver tumors involving the hepatic venous confluence or the retrohepatic IVC who underwent extended liver resection with vascular reconstruction.

METHODS

All pediatric patients undergoing major hepatectomy with reconstruction of an HV or the IVC over a 10-year study period were included. Preoperative imaging, surgical techniques, and short- and long-term postoperative data were analyzed.

RESULTS

From a total of 125 pediatric major hepatectomies, 17 children (15 hepatoblastoma, two undifferentiated embryonal sarcoma) underwent liver resection with vascular reconstruction of an HV or the IVC. In nine cases an HV was reconstructed, and in eight children, a partial resection of the IVC was performed. Total vascular exclusion of the liver was applied in 16/17 cases. No 90-day postoperative mortality, no major postoperative complication, and no local relapse occurred; 16/17 patients are alive without relapse at a median follow-up of 44 months (range 19-111).

CONCLUSION

This is the largest single-center series to report major hepatectomies with HV or IVC reconstruction in children. In specialized centers, these complex procedures are associated with excellent outcomes. Successful tumor resection can be achieved in selected cases even in locally advanced tumor stages.

摘要

背景

表现为中心性肿块且与所有三条肝静脉(HV)及下腔静脉(IVC)接触甚至侵犯的小儿肝脏肿瘤带来了重大的手术挑战。对于真正无法切除的肿瘤,可能需要进行肝移植,而扩大肝切除并进行血管重建则是一种保留器官的替代方法。

目的

本研究旨在介绍一个参考中心针对患有累及肝静脉汇合处或肝后下腔静脉的肝脏肿瘤并接受扩大肝切除及血管重建的儿童所采取的策略。

方法

纳入在10年研究期间接受了HV或IVC重建的扩大肝切除术的所有小儿患者。分析术前影像学、手术技术以及术后短期和长期数据。

结果

在总共125例小儿扩大肝切除术中,17例儿童(15例肝母细胞瘤,2例未分化胚胎性肉瘤)接受了HV或IVC血管重建的肝切除术。9例重建了HV,8例儿童进行了IVC部分切除术。16/17例患者应用了全肝血管阻断。术后90天无死亡,无重大术后并发症,无局部复发;16/17例患者存活且无复发,中位随访时间为44个月(范围19 - 111个月)。

结论

这是报道小儿HV或IVC重建扩大肝切除术的最大单中心系列研究。在专业中心,这些复杂手术的效果极佳。即使在局部晚期肿瘤阶段,部分病例也能成功实现肿瘤切除。

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