Nationwide survey of the outcomes of living donor liver transplantation for hepatoblastoma in Japan
Nationwide survey of the outcomes of living donor liver transplantation for hepatoblastoma in Japan
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DOI：
10.1002/lt.23803
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发表时间：
2014-03-01
影响因子：
4.6
通讯作者：
Uemoto, Shinji
中科院分区：
文献类型：
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作者：
Sakamoto, Seisuke;Kasahara, Mureo;Uemoto, Shinji
近年来，肝移植（LT）已越来越多地用于治疗不能切除的肝母细胞瘤（HB），并取得了可接受的结果。我们对因HB接受活体肝移植（LDLT）的病例进行了一项全国性调查，以评估其结局。39例患者（28例男性和11例女性，LDLT时的中位年龄为3.6岁）在2009年底接受了LDLT治疗HB，入组本研究。通过2011年的问卷调查（中位随访时间= 4.6年）从他们的病历中收集临床数据。13例患者（33.3%）在LDLT前有肝外病变。38例患者（97.4%）接受了化疗，15例（38.5%）在LDLT前接受了肝切除术。27例（69.2%）患者在LDLT后存活，无复发，12例（30.8%）患者复发。复发部位以肺最多（9例），其次为移植肝（6例）。从LDLT到复发的中位时间为5.5个月。9例肺转移中4例（44.4%）行手术切除，3例存活，无肿瘤复发。8例患者因肿瘤复发而死亡。多变量界标分析显示，独立的复发风险因素是诊断时甲胎蛋白（AFP）水平高[>= 500，000 ng/mL;风险比（HR）= 7.86，P = 0.010]，LDLT前存在肝外病变（HR = 3.82，P = 0.042），LDLT时AFP水平较高（>= 4000 ng/mL; HR = 9.19，P = 0.036）。3年和5年患者生存率分别为84.3%和77.3%。总之，在适当的时间安排LT，LDLT为HB儿童提供了一种有价值的替代治疗方法，效果良好。肝移植20：333 - 346，2014。(c)2013年AASLD。
Recently, liver transplantation (LT) has been increasingly performed for unresectable hepatoblastoma (HB) with acceptable results. We conducted a national survey of cases undergoing living donor liver transplantation (LDLT) for HB to evaluate their outcomes. Thirty-nine patients (28 males and 11 females with a median age at LDLT of 3.6 years) who had undergone LDLT for HB by the end of 2009 were enrolled in this study. The clinical data were collected from their medical records via a questionnaire survey in 2011 (median follow-up = 4.6 years). Thirteen patients (33.3%) had extrahepatic lesions before LDLT. Thirty-eight patients (97.4%) received chemotherapy, and 15 (38.5%) underwent hepatectomy before LDLT. Twenty-seven patients (69.2%) were alive without recurrence after LDLT, and 12 patients (30.8%) suffered from recurrence. The most common site of recurrence was the lung (9 cases), which was followed by the graft liver (6 cases). The median interval from LDLT to recurrence was 5.5 months. Four of the 9 cases (44.4%) with lung metastasis underwent surgical resection, and 3 were alive without any tumor recurrence. Eight patients died because of tumor recurrence. The multivariate landmark analysis revealed that the independent recurrence risk factors were a high alpha-fetoprotein (AFP) level at diagnosis [>= 500,000 ng/mL; hazard ratio (HR) = 7.86, P = 0.010], the presence of extrahepatic lesions before LDLT (HR = 3.82, P = 0.042), and a high AFP level at LDLT (>= 4000 ng/mL; HR = 9.19, P = 0.036). The actuarial 3- and 5-year patient survival rates were 84.3% and 77.3%, respectively. In conclusion, with appropriate timing for scheduled LT, LDLT provides a valuable alternative treatment with excellent results for children with HB. Liver Transpl 20:333-346, 2014. (c) 2013 AASLD.