Abstract：
Hepatoblastoma (HB) is a rare liver tumour, and its congenital counterpart (CHB) is even less frequent. CHB has a clinically challenging management and a generally perceived worse outcome. This study aims to review the literature on CHB to better define presentation, diagnosis, available treatments and management options. The analysis of outcomes suggests that a significant portion of mortality is unrelated to the malignant nature of the tumour. Key factors influencing overall outcomes were identified: mortality linked to the \'mass effect\' during both the prenatal (22%) and perinatal (32%) stages, as well as \'oncological\' mortality encompassing tumour and/or treatment-related factors (46%). Overall, after birth, CHB does not seem to confer a worse oncological prognosis per se, and should be managed similarly to older children, if patients are stable enough to undergo proper staging and treatment. A deeper knowledge and better outcomes would come from a large, homogeneous, collection of data possibly allowing a global protocol, focusing on a comprehensive management of CHB.
摘要：
肝母细胞瘤（HB）是一种罕见的肝脏肿瘤，其先天性对应物（CHB）甚至更不频繁。CHB具有临床上具有挑战性的管理和普遍感知的较差结果。本研究旨在回顾CHB的文献，以更好地定义演示文稿，诊断,可用的治疗和管理选择。结果分析表明，很大一部分死亡率与肿瘤的恶性性质无关。确定了影响总体结局的关键因素：在产前（22％）和围产期（32％）阶段与“质量效应”相关的死亡率。以及肿瘤死亡率，包括肿瘤和/或治疗相关因素(46%)。总的来说,出生后,CHB本身似乎并不意味着更糟糕的肿瘤预后，应该像年龄较大的孩子一样管理，如果患者足够稳定，可以进行适当的分期和治疗。更深入的知识和更好的结果将来自于一个大的，同质，可能允许全球协议的数据收集，专注于CHB的全面管理。
