Chen J C, Chen C C, Chen W J, Lai H S, Hung W T, Lee P H
Department of Surgery, National Taiwan University Hospital, Taipei.
J Pediatr Surg. 1998 Sep;33(9):1350-4. doi: 10.1016/s0022-3468(98)90005-7.
Hepatocellular carcinoma (HCC) in children was rarely reported and usually included with hepatoblastoma in most studies of pediatric liver malignancies despite different clinical behaviors. The authors report their experience in pediatric HCC and discuss its differences from adult HCC.
A retrospective review of radiographic, laboratory, pathological, and therapeutic data in 55 children with HCC was performed. The liver function was graded by modified Child's classification. Kaplan-Meier survival curves in various therapeutic and Child's groups were plotted, and log-rank test was used to detect differences among survival curves.
Although children with HCC mostly presented with advanced disease at diagnosis, disturbances of liver function were unremarkable. Sixty-eight percent of cases concurred with liver cirrhosis. The median survivals for resectable, chemotherapeutic, and untreated HCCs were 23, 3, and 2 months, respectively. Resectable HCC significantly posed a much better prognosis. However, the resectability was unsatisfactory (18.2%). Resection was limited because of anatomic unfeasibility including bilateral involvement (62.5%), portal vein thrombi (41.7%), distant metastasis (29.1%), para-aortic lymphadenopathy (18.8%), inferior vena cava thrombi (16.7%), and hilar invasion (6.3%). Distant metastasis was the most ominous for survival in children with unresectable HCC.
HCC behaved somewhat differently between children and adults. Surgical resection represented the best hope of long-term survival. The outcome in children could not keep up with that in adults because of a diagnostic delay. Hence, alpha-fetoprotein and sonography screening in carrier children should be worthwhile.
儿童肝细胞癌（HCC）鲜有报道，在大多数小儿肝脏恶性肿瘤研究中，尽管其临床行为不同，但通常与肝母细胞瘤归为一类。作者报告了他们在小儿HCC方面的经验，并讨论了其与成人HCC的差异。
对55例儿童HCC患者的影像学、实验室、病理和治疗数据进行回顾性分析。肝功能采用改良Child分级法进行评估。绘制不同治疗组和Child分级组的Kaplan-Meier生存曲线，并采用对数秩检验检测生存曲线之间的差异。
尽管儿童HCC在诊断时大多表现为晚期疾病，但肝功能紊乱并不明显。68%的病例合并肝硬化。可切除、化疗和未治疗的HCC患者的中位生存期分别为23个月、3个月和2个月。可切除的HCC预后明显更好。然而，可切除率并不理想（18.2%）。由于解剖学上不可行，包括双侧受累（62.5%）、门静脉血栓形成（41.7%）、远处转移（29.1%）、主动脉旁淋巴结肿大（18.8%）、下腔静脉血栓形成（16.7%）和肝门侵犯（6.3%），手术切除受到限制。远处转移对不可切除的儿童HCC患者的生存最为不利。
儿童和成人的HCC表现有所不同。手术切除是长期生存的最大希望。由于诊断延迟，儿童的治疗效果不如成人。因此，对携带相关基因的儿童进行甲胎蛋白和超声筛查是值得的。
