Hepatoblastoma with a low serum alpha-fetoprotein level at diagnosis: The SIOPEL group experience
Hepatoblastoma with a low serum alpha-fetoprotein level at diagnosis: The SIOPEL group experience
复制标题
DOI：
10.1016/j.ejca.2007.11.022
复制
发表时间：
2008-03-01
影响因子：
8.4
通讯作者：
Perilongo, Giorgio
中科院分区：
文献类型：
--
作者：
De Ioris, Maretta;Brugieres, Laurence;Perilongo, Giorgio
研究目的：目的：探讨肝母细胞瘤（HCC）诊断时血清甲胎蛋白（AFP）水平低的患者的临床特点，患者与方法：纳入21例SIOPEL试验患者，诊断时血清AFP < 100 ng/ml。所有15例患者的切片与可用的组织学材料集中review.Results：中位年龄：10个月。诊断时的疾病扩展：PRETEXT组：II（3例患者）、111（10例患者）和IV（8例患者）。肝外扩展：8例患者。多灶性肿瘤：8例患者。审查时的组织学：全上皮亚型：11/15例患者，包括9例小细胞未分化组织学。结果：仅9例患者获得部分缓解，16例死亡。中位生存期：4.4个月。2年总生存期：结论：本研究明确将诊断时血清AFP低的肝母细胞瘤患者确定为诊断时疾病广泛、化疗反应差和预后差的高危亚组。(c)2007爱思唯尔有限公司保留所有权利。
Aim of the study: To investigate the characteristics of patients with hepatoblastoma and low serum alpha-fetoprotein (AFP) at diagnosis.Patients and methods: Inclusion of all 21 patients accrued onto SIOPEL trials, whose serum AFP was < 100 ng/ml at diagnosis. Slides of all 15 patients with available histological material were centrally reviewed.Results: Median age: 10 months. Disease extension at diagnosis: PRETEXT group: II (3 patients), 111 (10 patients) and IV (8 patients). Extra-hepatic extension: 8 patients. Multifocal tumour: 8 patients. Histology at review: wholly epithelial subtype: 11/15 patients including nine with a small-cell undifferentiated histology. outcome: only 9 patients achieved a partial response and 16 died. Median survival: 4.4 months. Two-year overall survival: 24% (confidence interval 10-45%).Conclusion: This study clearly identifies patients with hepatoblastoma and low serum AFP at diagnosis as a high-risk subgroup with extensive disease at diagnosis, poor response to chemotherapy and a poor outcome. (c) 2007 Elsevier Ltd. All rights reserved.