Romano Fabrizio, Stroppa Paola, Bravi Michela, Casotti Valeria, Lucianetti Alessandro, Guizzetti Michela, Sonzogni Aurelio, Colledan Michele, D'Antiga Lorenzo
Department of Surgical Science, S. Gerardo Hospital, Milan, Italy.
Pediatr Transplant. 2011 Sep;15(6):573-9. doi: 10.1111/j.1399-3046.2011.01528.x. Epub 2011 Jul 29.
The outcome of HCC after transplantation (OLT) in children is not well known. Unfavorable features based on adult reports may lead to contraindicate OLT even in children. We reviewed a cohort of children with cirrhosis and HCC to evaluate their outcome after primary transplantation. We considered children with cirrhosis and HCC who had a primary OLT. We retrospectively recorded demographic, medical and surgical features, and MC as predictors of outcome. Among 456 children transplanted in the last 15 yr, 10 (2%), median age at diagnosis 1.8 yr (range 0.5-7.2), had HCC in biliary atresia (3), BSEP deficiency (3), tyrosinemia type 1 (2), complications of choledocal cyst and glycogen storage disease type IV (1 each). At HCC discovery, median AFP was 2322 ng/mL (3-35,000), high or rising in 9/10 patients. Six patients were outside the MC. Median time on the waiting list was 38 days (1-152). Two patients died from early complications of OLT. In the other eight patients, there was no tumor recurrence after a median follow-up of four yr. Children with cirrhosis may develop HCC at a very young age. The outcome appears excellent even outside MC. Primary liver transplantation is advisable for children with cirrhosis, HCC, and no extrahepatic disease.
儿童肝移植（OLT）后肝细胞癌（HCC）的预后情况尚不明确。基于成人报告的不良特征可能导致即使在儿童中也禁忌进行OLT。我们回顾了一组肝硬化合并HCC的儿童，以评估他们初次移植后的预后。我们纳入了接受初次OLT的肝硬化合并HCC儿童。我们回顾性记录了人口统计学、医学和手术特征以及作为预后预测指标的米兰标准（MC）。在过去15年接受移植的456名儿童中，10名（2%）诊断时的中位年龄为1.8岁（范围0.5 - 7.2岁），患有HCC，病因包括胆道闭锁（3例）、BSEP缺乏症（3例）、1型酪氨酸血症（2例）、胆总管囊肿并发症和IV型糖原贮积病（各1例）。发现HCC时，中位甲胎蛋白（AFP）为2322 ng/mL（3 - 35,000），9/10的患者AFP高或呈上升趋势。6名患者不符合MC标准。等待移植的中位时间为38天（1 - 152天）。2名患者死于OLT的早期并发症。在其他8名患者中，中位随访4年后无肿瘤复发。肝硬化儿童可能在非常年幼时就发生HCC。即使不符合MC标准，预后似乎也很好。对于患有肝硬化、HCC且无肝外疾病的儿童，建议进行原位肝移植。
