Abstract:

Objective To summarize the clinical effect and prognosis of liver transplant in children with hepatoblastoma（HB）. Methods A retrospective analysis was performed on 8 cases of liver transplantation（LT）with HB in Department of Hematology and Oncology of Shanghai Children’s Hospital from January 2014 to December 2020. Results Eight patients underwent LT，accounting for 12.1% of HB patients during the same period，there were 3 males and 5 females，with a median age of 54（30 ～ 130）months，the median age at transplant was 67.5（36 ～ 175）months. According to PRETEXT stage ：3 patients were in stage Ⅲ and 5 in stage Ⅳ，with 1 involving hepatic vein and4 involving portal vein. Lung metastasis was found in 2 cases. According to POST-TEXT stage ：6 patients were in stage Ⅲ and 2 in stage Ⅳ . The median AFP value was 55 450（23 950 ～＞ 121 000）ng/ml at diagnosis and 242（6.78 ～ 58025）ng/ml before LT，respectively. The median declines in AFP was 99.7%（52.0% ～ 99.9%），and The median AFP value was 6.02（3.58 ～ 15 015）ng/ml after LT. All patients were treated with chemotherapy with a median course of 6 course before LT and 3 courses after LT. The median follow-up time was 10.5（1 ～ 42）months，CR occurred in 6 cases，PR occurred in 1 case and 1 patient died. The mean survival time was（36.9±4.8）months.The overall survival rate was 87.5%. Conclusion The treatment of pediatric hepatoblastoma is a comprehensive treatment mode. Total liver resection combined with LT can achieve satisfactory results，but refractory HB and recurrence are still the difficulties in the current treatment.

Key words:

 , Child , Hepatoblastoma, Liver transplant, Alpha-fetoprotein

摘要：

目 的 总 结 肝 移 植 治 疗 儿 童 肝 母 细 胞 瘤（hepatoblastoma，HB） 的 临 床 疗 效 及 预 后。方法 回顾性分析 2014 年 1 月至 2020 年 12 月上海市儿童医院明确诊断为 HB 并接受肝移植治疗的 8 例患者的临床资料。结果 本组共 8 例行肝移植治疗的 HB 患者，占同期 HB 患者比例 12.1%，男性 3 例，女性 5 例。中位发病年龄为 54（30 ～ 130）个月，中位移植年龄为 67.5（36 ～ 175）个月。PRETEXT 分期Ⅲ期 3 例，Ⅳ期 5 例。肝静脉受累 1 例，门静脉受累 4 例。肺部转移 2 例。POST-TEXT 分期Ⅲ期 6 例，Ⅳ期 2 例。诊断时 AFP 中位值为 55 450（23 950 ～＞ 121 000）ng/ml。移植前 AFP 中位值为 242（6.78 ～ 58025）ng/ml。AFP 下降比例中位值为 99.7%（52.0% ～ 99.9%）。移植后 AFP 中位值为 6.02（3.58 ～ 15 015）ng/ml。所有患者均行术前化疗，术前化疗中位疗程 6 次。原发肝移植 6 例。挽救性肝移植 2 例。术后化疗 6 例。术后化疗中位疗程 3 次。中位随访时间为 10.5（1 ～ 42）个月，CR 为 6 例，PR 为 1 例，死亡 1 例。平均生存时间为（36.9±4.8）个月。总生存率为 87.5%。结论 儿童肝母细胞瘤的治疗是多种方式结合的综合治疗模式，全肝切除联合肝移植可取得满意的疗效，难治、复发患者仍是目前治疗的难点。

关键词:

儿童 , 肝母细胞瘤 , 肝移植 , 甲胎蛋白