Department of Public Health and Center of Biostatistics, College of Medicine, Chang Gung University, Taoyuan, Taiwan.
J Formos Med Assoc. 2011 May;110(5):322-5. doi: 10.1016/S0929-6646(11)60048-X.
BACKGROUND/PURPOSE: Hepatoblastoma is the most common malignant liver tumor in children. Comparative studies have elucidated the optimal pre- or postoperative chemotherapeutic regimens. The aim of this study was to investigate the prognostic significance of baseline tumor characteristics for overall survival and disease-free survival in children with hepatoblastoma.
There were 19 male and 16 female children with a median age of 19 months at diagnosis (range: 1-169 months) in our institution between February 1990 and June 2009. We reviewed the clinical presentation, serum α-fetoprotein level at diagnosis, histological subtype, treatment, and outcomes.
Twenty-seven patients (78%) underwent neoadjuvant chemotherapy. The majority of patients subsequently underwent either hemihepatectomy (56%) or bisegmentectomy (16%). Only six patients underwent extended hepatic resection, and one of them required rescue liver transplantation. During follow-up, six patients died of progressive disease and two of perioperative mortality. Four of the six who died had pulmonary metastases at the time of diagnosis or follow-up. The median survival time was 28 months (range: 1-181 months). Five-year overall survival was 67.7% (95% confidence interval: 52.0-87.8%) and disease-free survival was 60.2% (95% confidence interval: 41.9-86.5%).
The potential down-staging effect of neoadjuvant chemotherapy on hepatoblastoma might facilitate remission and convert unresectable tumors into operable ones.
背景/目的：肝母细胞瘤是儿童中最常见的恶性肝脏肿瘤。对比研究已经阐明了最佳的术前或术后化疗方案。本研究旨在探讨基线肿瘤特征对肝母细胞瘤患儿总生存和无病生存的预后意义。
在我们机构，1990 年 2 月至 2009 年 6 月期间，有 19 名男性和 16 名女性患儿，中位诊断年龄为 19 个月（范围：1-169 个月）。我们回顾了临床表现、诊断时的血清甲胎蛋白水平、组织学亚型、治疗和结局。
27 例（78%）患儿接受了新辅助化疗。大多数患儿随后接受了半肝切除术（56%）或双段切除术（16%）。只有 6 例患儿接受了扩大肝切除术，其中 1 例需要进行挽救性肝移植。在随访期间，6 例患儿死于疾病进展，2 例患儿死于围手术期。死亡的 6 例患儿中有 4 例在诊断或随访时已有肺部转移。中位生存时间为 28 个月（范围：1-181 个月）。5 年总生存率为 67.7%（95%置信区间：52.0-87.8%），无病生存率为 60.2%（95%置信区间：41.9-86.5%）。
新辅助化疗对肝母细胞瘤的潜在降期作用可能有助于缓解疾病，并将不可切除的肿瘤转化为可切除的肿瘤。
