Abstract：
BACKGROUND: Patients with hepatoblastoma (HB) have a higher risk of congenital heart defects (CHD). There is limited literature on the management and outcomes of these patients. The purpose of this study was to identify demographics and outcomes of these patients in a single tertiary referral center.
METHODS: An Institutional Review Board (IRB)-approved retrospective chart review of patients with newly diagnosed HB from October 2004 to January 2021 was performed. CHD was defined as the presence of a septal defect, patent ductus arteriosus, pulmonary atresia, or bicuspid aortic valve. Chi-square and t-test were utilized for statistical analyses.
RESULTS: Of the 151 patients diagnosed with HB during the study timeframe, 29 patients were found to have CHD. Five-year overall survival (OS) for non-CHD HB patients was 81.9% compared to 68.9% in the CHD cohort (p = .12). The 5-year OS for patients without surgically intervened CHD was 63.6% compared to 70.5% for those with surgically repaired CHD (p = .88). Pre-treatment extent of tumor IV was present more often in patients with HB and CHD who passed away (6/9, 66.7%) compared to those who survived (3/16,18.8%, p = .01).
CONCLUSIONS: Patients with HB and CHD have similar survival compared to those without CHD. Our data support that patients with HB and CHD should be treated with curative intent including cardiac surgical intervention, medical oncology therapy, and oncological surgery for their HB.
METHODS: An Institutional Review Board (IRB)-approved retrospective chart review of patients with newly diagnosed HB from October 2004 to January 2021 was performed. CHD was defined as the presence of a septal defect, patent ductus arteriosus, pulmonary atresia, or bicuspid aortic valve. Chi-square and t-test were utilized for statistical analyses.
RESULTS: Of the 151 patients diagnosed with HB during the study timeframe, 29 patients were found to have CHD. Five-year overall survival (OS) for non-CHD HB patients was 81.9% compared to 68.9% in the CHD cohort (p = .12). The 5-year OS for patients without surgically intervened CHD was 63.6% compared to 70.5% for those with surgically repaired CHD (p = .88). Pre-treatment extent of tumor IV was present more often in patients with HB and CHD who passed away (6/9, 66.7%) compared to those who survived (3/16,18.8%, p = .01).
CONCLUSIONS: Patients with HB and CHD have similar survival compared to those without CHD. Our data support that patients with HB and CHD should be treated with curative intent including cardiac surgical intervention, medical oncology therapy, and oncological surgery for their HB.
摘要：
背景：肝母细胞瘤（HB）患者患先天性心脏缺陷（CHD）的风险更高。关于这些患者的治疗和结果的文献有限。这项研究的目的是确定单个三级转诊中心中这些患者的人口统计学和结局。
方法：对2004年10月至2021年1月新诊断的HB患者进行了机构审查委员会（IRB）批准的回顾性图表审查。CHD被定义为存在间隔缺损，动脉导管未闭,肺动脉闭锁,或二叶主动脉瓣。采用卡方检验和t检验进行统计分析。
结果：在研究时间段内诊断为HB的151例患者中，29例患者被发现患有冠心病。非CHDHB患者的五年总生存率（OS）为81.9％，而CHD队列为68.9％（p=.12）。没有手术介入的冠心病患者的5年OS为63.6%，而手术修复的冠心病患者为70.5%(p=0.88)。与存活患者(3/16,18.8%,p=.01）。
结论：患有HB和CHD的患者与没有CHD的患者具有相似的生存率。我们的数据支持HB和CHD患者应进行治愈性治疗，包括心脏手术干预。肿瘤内科治疗,以及他们HB的肿瘤手术.
方法：对2004年10月至2021年1月新诊断的HB患者进行了机构审查委员会（IRB）批准的回顾性图表审查。CHD被定义为存在间隔缺损，动脉导管未闭,肺动脉闭锁,或二叶主动脉瓣。采用卡方检验和t检验进行统计分析。
结果：在研究时间段内诊断为HB的151例患者中，29例患者被发现患有冠心病。非CHDHB患者的五年总生存率（OS）为81.9％，而CHD队列为68.9％（p=.12）。没有手术介入的冠心病患者的5年OS为63.6%，而手术修复的冠心病患者为70.5%(p=0.88)。与存活患者(3/16,18.8%,p=.01）。
结论：患有HB和CHD的患者与没有CHD的患者具有相似的生存率。我们的数据支持HB和CHD患者应进行治愈性治疗，包括心脏手术干预。肿瘤内科治疗,以及他们HB的肿瘤手术.
