Department of Pediatrics, Division of Pediatric Hepatology, St. Louis Children's Hospital, 660 S. Euclid Avenue, Campus Box 8112, St. Louis, MO, 63110, USA.
Department of Surgery, Division of Abdominal Transplant Surgery, Washington University in St Louis, St. Louis, MO, USA.
J Gastrointest Cancer. 2021 Jun;52(2):547-556. doi: 10.1007/s12029-020-00421-z.
Hepatoblastoma (HB) is a rare childhood malignancy with hepatic resection (HR) or liver transplantation (LT) providing the best chance of cure. In this study, we analyze the National Cancer Database lacks (NCDB) to compare outcomes following HR and LT for HB.
Review of the US experience with surgical (HR and LT) management of pediatric (< 18 years) HB over the last decade (2004-2014) using data extracted from the NCDB.
A total of 628 children underwent surgical treatment for HB during the study period: HR in 525 (83.6%) and LT in 103 (16.4%). The two groups were comparable for age, sex, race, tumor size, and metastatic disease at initial diagnosis. LT group had significantly higher number of patients with bilobar disease (40 vs 21%; p < 0.001), longer median time from diagnosis to surgery (120 vs 78 days; p < 0.001), and longer post-operative length of stay (LOS) (14 vs 6 days; p < 0.001). There were no differences in rates of 30-day readmission and 30- or 90-day mortality between groups. Both groups had comparable 5-year overall survival (OS) (84.1% HR vs 80.0% LT; p = 0.4). Univariate analysis identified metastatic disease at initial presentation (HR 2.56, CI 1.51-4.35) and age ≥ 4 years (HR 2.68, CI 1.5-4.7) as risk factors for worse overall 5-yr OS, while administration of adjuvant chemotherapy was associated with improved 5-yr OS (92.3% with chemo vs 85.4% without chemo; HR 0.51, CI 0.31-0.84).
The outcome of HB has improved compared with historical controls. Age at presentation, metastatic disease, and post-operative chemotherapy impact outcomes.
肝母细胞瘤（HB）是一种罕见的儿童恶性肿瘤，肝切除术（HR）或肝移植（LT）是治愈的最佳机会。在这项研究中，我们分析了国家癌症数据库（NCDB），以比较 HB 接受 HR 和 LT 后的结果。
回顾了过去十年（2004-2014 年）美国使用从 NCDB 提取的数据对小儿（<18 岁）HB 进行手术（HR 和 LT）管理的经验。
研究期间共有 628 名儿童接受了 HB 的手术治疗：HR 为 525 例（83.6%），LT 为 103 例（16.4%）。两组在年龄、性别、种族、肿瘤大小和初始诊断时的转移性疾病方面具有可比性。LT 组患有双侧疾病的患者比例明显更高（40%比 21%；p<0.001），从诊断到手术的中位时间更长（120 比 78 天；p<0.001），术后住院时间更长（14 比 6 天；p<0.001）。两组 30 天再入院率和 30 天或 90 天死亡率无差异。两组 5 年总生存率（OS）均相似（HR 为 84.1%，LT 为 80.0%；p=0.4）。单因素分析确定初始表现时存在转移性疾病（HR 2.56，CI 1.51-4.35）和年龄≥4 岁（HR 2.68，CI 1.5-4.7）是总 5 年 OS 较差的危险因素，而辅助化疗的应用与改善的 5 年 OS 相关（接受化疗的 92.3%与未接受化疗的 85.4%；HR 0.51，CI 0.31-0.84）。
与历史对照相比，HB 的结果有所改善。就诊时的年龄、转移性疾病和术后化疗影响结果。
