肝移植治疗儿童肝母细胞瘤结果分析

Treatment outcome of liver transplant in children with hepatoblastoma

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。

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 and 4 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.