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儿童肝恶性肿瘤的活体肝移植。

Living donor liver transplantation for hepatic malignancies in children.

机构信息

Pediatric and Transplantation Surgery, Cliniques Universitaires Saint-Luc, Brussels, Belgium.

Pediatric Hepatology and Gastroenterology, Cliniques Universitaires Saint-Luc, Brussels, Belgium.

出版信息

Pediatr Transplant. 2021 Nov;25(7):e14047. doi: 10.1111/petr.14047. Epub 2021 Jun 2.

Abstract

BACKGROUND

Living donor liver transplantation is a treatment option for unresectable hepatic tumors in children.

METHODS

We enrolled 45 living donor transplantations performed between 1993 and 2018 for liver malignacies, which included hepatoblastoma (n = 33), hepatocellular carcinoma (n = 10), hepatic angiosarcoma (n = 1), and rhabdomyosarcoma (n = 1).

RESULTS

No mortality or major morbidities were encountered in any donor, and the complication rate was 9%. In the hepatoblastoma group, 5-year overall and event-free survival rate in recipients was 87.4% and 75.8%, respectively, and mortality was significantly higher in patients after rescue transplantation (p = .001). Inferior vena cava replacement in these recipients appeared to be associated with reduced mortality (p = .034), but this was not confirmed when rescue patients were excluded (p = .629). In hepatocellular carcinoma group, both 5-year overall and event-free survival rates were 75.4% each, and invasion of hepatic veins was significantly associated with increased risk of recurrence and death (p = .028). The patient with rhabdomyosarcoma died from EBV-induced lymphoma 2 months after transplantation. The patient with angiosarcoma was in complete remission at the last follow-up. Overall, 5-year graft survival rate was 81.3%, and one patient underwent re-transplantation due to chronic rejection.

CONCLUSIONS

Pediatric oncological liver transplantation has become a key player in the management of malignancies with cancer cure in 84% of patients in this series. Living donor liver transplantation for pediatric recipients with unresectable tumors might be a beneficial surgical option, which is technically safe for donors and recipients, thus, allowing timely planning according to chemotherapy protocols.

摘要

背景

活体肝移植是儿童不可切除肝肿瘤的一种治疗选择。

方法

我们纳入了 1993 年至 2018 年间进行的 45 例因肝脏恶性肿瘤而进行的活体供肝移植,包括肝母细胞瘤(n=33)、肝细胞癌(n=10)、肝血管肉瘤(n=1)和横纹肌肉瘤(n=1)。

结果

在任何供体中均未发生死亡或重大并发症,并发症发生率为 9%。在肝母细胞瘤组中,受者的 5 年总生存率和无事件生存率分别为 87.4%和 75.8%,挽救性移植后的死亡率显著升高(p=0.001)。在这些受者中,下腔静脉置换术似乎与降低死亡率相关(p=0.034),但当排除挽救性患者时,这并未得到证实(p=0.629)。在肝细胞癌组中,5 年总生存率和无事件生存率均为 75.4%,肝静脉侵犯与复发和死亡风险增加显著相关(p=0.028)。患有横纹肌肉瘤的患者在移植后 2 个月因 EBV 诱导的淋巴瘤死亡。患有血管肉瘤的患者在最后一次随访时处于完全缓解状态。总体而言,5 年移植物存活率为 81.3%,1 例患者因慢性排斥反应而再次接受移植。

结论

在本系列中,儿科肿瘤肝脏移植已成为管理恶性肿瘤的重要手段,使 84%的患者获得癌症治愈。对于患有不可切除肿瘤的儿科受者,活体供肝移植可能是一种有益的手术选择,对供者和受者来说在技术上是安全的,从而可以根据化疗方案进行及时的计划。

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