316 例 3 岁以下儿童肝母细胞瘤的临床特征和预后因素-14 年回顾性单中心研究。
Clinical characteristics and prognostic factors of hepatoblastoma in 316 children aged under 3 years - a 14-year retrospective single-center study.
机构信息
Department of Pediatrics, Beijing Tongren Hospital Capital Medical University, No. 1 Dongjiaominxiang Dongcheng District, Beijing, 100730, China.
出版信息
BMC Pediatr. 2021 Apr 13;21(1):170. doi: 10.1186/s12887-021-02630-2.
BACKGROUND
The aim of the present study was to summarize the clinical characteristics of in children aged under 3 years and analyze the related factors affecting the prognosis.
METHODS
The clinical data of 316 children aged under 3 years (192 males and 124 females) who were admitted to Beijing Tongren Hospital with a pathological diagnosis of HB between May 2005 and May 2019 were analyzed retrospectively. The factors influencing the therapeutic effects on and survival of HB in children with HB were analyzed.
RESULTS
(1) The median age of the enrolled patients was 1.45 years. The most common initial symptom was an abdominal mass (69.0%). The average serum alpha-fetoprotein (AFP) level and platelet count at the initial visit were (97,406.5 ± 5022.8) ng/mL and (418 ± 206) × 10/L, respectively. The epithelial type was the main pathological type (51.9%). According to the PRETEXT preoperative typing system, the most common stage was stage III (57.0%), whereas according to the postoperative Evans staging system, the most common stage was stage IV (41.8%). At the initial visit, 62 cases (19.6%) had vascular invasion, 52 cases (16.5%) had extrahepatic tumor extension, and 20 cases (6.3%) had tumor rupture. Distant metastasis occurred in 132 cases, and the most common metastatic site was the lung (80.3%). The incidence in East China was relatively high (35.4%). (2) The children were followed up until May 2020 (the median follow-up duration was 62 months). It was found that 194 patients had complete remission and 62 had partial remission. The Kaplan-Meier survival analysis showed that the overall survival was 95.3, 88.2, and 79.8% at 1 year, 3 years, and 5 years, respectively, and the event-free survival was 91.1, 83.2, and 75.1%, respectively. The Cox regression analysis showed that AFP level, platelet count, PRETEXT IV, vascular invasion, and distant metastasis at the initial visit were independent risk factors for the prognosis of children with HB (p < 0.05 in all).
CONCLUSION
The prognosis of HB was correlated with the AFP level, platelet count, PRETEXT staging, vascular invasion, and distant metastasis at initial diagnosis.
背景
本研究旨在总结 3 岁以下儿童 HB 的临床特征，并分析影响预后的相关因素。
方法
回顾性分析 2005 年 5 月至 2019 年 5 月期间因病理诊断为 HB 而在北京同仁医院住院的 316 例 3 岁以下儿童的临床资料。分析影响 HB 患儿治疗效果及生存的相关因素。
结果
（1）纳入患者的中位年龄为 1.45 岁。最常见的首发症状为腹部包块（69.0%）。初诊时血清甲胎蛋白（AFP）水平和血小板计数平均值分别为（97406.5±5022.8）ng/mL和（418±206）×10/L。上皮型为主要病理类型（51.9%）。根据 PRETEXT 术前分型系统，最常见的分期为 III 期（57.0%），而根据术后 Evans 分期系统，最常见的分期为 IV 期（41.8%）。初诊时 62 例（19.6%）有血管侵犯，52 例（16.5%）有肝外肿瘤播散，20 例（6.3%）有肿瘤破裂。132 例发生远处转移，最常见的转移部位为肺（80.3%）。华东地区发病率相对较高（35.4%）。（2）患儿随访至 2020 年 5 月（中位随访时间 62 个月）。结果显示，194 例患儿完全缓解，62 例部分缓解。Kaplan-Meier 生存分析显示，患儿的总生存率分别为 1 年、3 年和 5 年时的 95.3%、88.2%和 79.8%，无事件生存率分别为 1 年、3 年和 5 年时的 91.1%、83.2%和 75.1%。Cox 回归分析显示，初诊时 AFP 水平、血小板计数、PRETEXT Ⅳ期、血管侵犯和远处转移是影响 HB 患儿预后的独立危险因素（p 值均<0.05）。
结论
HB 的预后与 AFP 水平、血小板计数、PRETEXT 分期、血管侵犯和初诊时远处转移有关。
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