An analysis of the outcomes in living donor liver transplantation for pediatric malignant hepatic tumors using nationwide survey data in Japan

An analysis of the outcomes in living donor liver transplantation for pediatric malignant hepatic tumors using nationwide survey data in Japan
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DOI:
10.1111/tri.13924
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发表时间:
2021-06-22
影响因子:
3.1
通讯作者:
Ohdan, Hideki
Ohdan, Hideki
中科院分区:
医学3区
文献类型:
--
作者:
Uchida, Hajime;Sakamoto, Seisuke;Ohdan, Hideki

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在日本,儿童恶性肝肿瘤(MHTs)非常罕见,约占儿童肝移植(LT)候选人的5%。我们对1990年10月至2018年4月期间接受活体供者LT治疗MHT的儿科患者进行了一项全国性调查。在本研究期间,共有116名儿童因MHT接受了LT:100例肝母细胞瘤(HBL),10例肝细胞癌(HCC)和6例其他MHT。HBL患者的5年总生存率为81.3%,HCC为60.0%,其他MHT为80.0%(P = 0.047)。在HBL患者中,接受原发性LT的患者和因肿瘤复发接受挽救性LT的患者的1年和5年患者生存率无显著差异(89.7%,81.6% vs. 88.0%,76%; P = 0.526)。HBL LT后的5年总生存率从1996-2008年的63.2%显著改善至2009-2018年的89.8%(P = 0.018)。肝移植前有无肺转移对长期生存率无显著影响(P = 0.742)。5名HCC患者死亡,其中2名不符合米兰标准。总之,LT治疗儿童MHT,特别是HBL,是一种有价值的治疗选择。
Malignant hepatic tumors (MHTs) in children are rare and account for approximately 5% of candidates for pediatric liver transplantation (LT) in Japan. We conducted a national survey of pediatric patients undergoing living donor LT for MHTs between October 1990 and April 2018. In total, 116 children underwent LT for MHTs during this study period: 100 hepatoblastomas (HBLs), 10 hepatocellular carcinomas (HCCs), and six other MHTs. The overall patient survival rate at 5 years was 81.3% for HBL, 60.0% for HCC, and 80.0% for other MHTs (P = 0.047). In patients with HBL, there was no significant difference in the 1- and 5-year patient survival rates between patients undergoing primary LT and those who received salvage LT for tumor recurrence (89.7%, 81.6% vs. 88.0%, 76%; P = 0.526). The 5-year overall survival rate after LT for HBL significantly improved from 63.2% in 1996-2008 to 89.8% in 2009-2018 (P = 0.018). The presence of lung metastasis before LT had no significant influence on the long-term survival (P = 0.742). Five patients with HCC died, including two who fell outside the Milan criteria. In conclusion, LT for pediatric MHTs, especially HBL, is a valuable treatment option for select patients.