肝移植治疗儿童胆道闭锁合并肝脏恶性肿瘤4例报告

Liver transplantation for biliary atresia with hepatic malignant tumor:report of 4 cases

目的探讨肝移植治疗儿童胆道闭锁(biliary atresia,BA)合并肝脏恶性肿瘤的临床疗效及预后。方法2015年1月1日—2019年12月31日期间天津市第一中心医院儿童器官移植科的儿童肝移植患者中,有4例原发疾病为胆道闭锁合并肝恶性肿瘤,回顾性分析这4例患者的临床资料。结果4例患者中BA合并肝母细胞瘤(hepatoblastoma,HB)3例,BA合并肝细胞癌(hepatocellular carcinoma,HCC)1例,所纳入的患者术前均诊断BA,其中1例术前诊断为BA合并HB,其余3例为肝移植术后病理诊断为BA合并HB或HCC。4例患者肝移植术后均顺利恢复,术后中位随访时间为25.5个月,4例患者全部存活,随访期内均未发现肿瘤复发。结论肝移植是BA合并肝脏恶性肿瘤的有效治疗方式,对于低风险患者,移植术后未行辅助化疗的近期疗效确切,远期疗效尚需进一步研究。

Objective To investigate the clinical efficacy and prognosis of liver transplantation in children with biliary atresia combined with liver cancer.Methods From January 1,2015 to December 31,2019,there were 4 cases of biliary atresia combined with liver cancer in the Tianjin First Central Hospital.The clinical data of these 4 cases were analyzed retrospectively.Results Among the 4 biliary atresia patients,there were 3 cases complicated with hepatoblastoma and 1 case complicated with hepatocellular carcinoma.All the patients were diagnosed with biliary atresia preoperatively,1 case was diagnosed with hepatoblastoma preoperatively,and the other 3 cases were diagnosed with hepatoblastoma or hepatocellular carcinoma postoperatively.The median follow-up time was 25.5 months.All the 4 patients survived and no recurrence was found during the follow-up period.Conclusion Liver transplantation is an effective treatment for biliary atresia combined with liver cancer.For low-risk patients,the short-term effect without adjuvant chemotherapy after transplantation is definite,and the long-term outcomes need to be studied further.