肝细胞腺瘤(HCA)是罕见的良性肝肿瘤。儿童和成人的诱发因素和并发症发生率似乎有所不同。在本研究中,我们旨在系统地表征儿科HCA并确定其病程,并发症,和管理。病史,临床症状,成像,组织病理学,通过对已发表的文献进行系统和全面的审查,收集了HCA儿童的遗传学。本研究共纳入316名HCA儿童。HCA的诊断主要是女孩(59.3%),平均年龄为11.5(范围0-17.7)岁。大多数(83.6%)的HCA发生在患有易感疾病的儿童中,其中糖原贮积病是最常见的,其次是门体分流和MODY3(年轻3型成熟型糖尿病)。这些疾病中的每一种都导致明确的HCA分子模式。随着时间的推移,显著数量的HCA或者是出血(24.7%)或者是转化的(14.8%)。HCA转化在患有门体分流的儿童和β-连环蛋白突变的HCA中明显更频繁,而暴露于激素和病变较大的儿童出血更频繁。管理主要受任何易感条件和病变数量的指导。因此,血管分流器在可能的情况下关闭,同时切除复杂的病变。肝移植使治疗腺瘤病成为可能,以及任何潜在的疾病。在理解遗传和/或畸形贡献方面的进展,这在儿科HCA中似乎很重要,提供了对肿瘤发病机制的见解,并将进一步指导患者的监测和管理。
Hepatocellular adenomas (HCAs) are rare benign liver tumours. Predisposing factors and complication rates appear to differ among children and adults. In the present study, we aimed to systematically characterise paediatric HCAs and determine their course, complications, and management. Medical history, clinical symptoms, imaging, histopathology, and genetics of children with HCAs were collected through a systematic and comprehensive
review of the published literature. A total of 316 children with HCAs were included in the present study. HCAs were diagnosed primarily in girls (59.3%) and at a mean age of 11.5 (range 0-17.7) years. The majority (83.6%) of HCAs occurred in children with predisposing diseases, of which glycogen storage disease was the most common, followed by portosystemic shunts and MODY3 (maturity-onset diabetes of the young type 3). Each of these diseases leads to a well-defined HCA molecular pattern. A significant number of HCAs either bled (24.7%) or transformed (14.8%) over time. HCA transformation was significantly more frequent in children with portosystemic shunts and in β-catenin-mutated HCAs, while haemorrhages were more frequent in children exposed to hormones and those with larger lesions. Management was primarily guided by any predisposing conditions and the number of lesions. Therefore, vascular shunts were closed when possible, while complicated lesions were resected. Liver transplantation has made it possible to treat adenomatosis, as well as any underlying diseases. Progress in understanding genetic and/or malformative contributions, which appear to be significant in paediatric HCAs, have provided insights into tumour pathogenesis and will further guide patient surveillance and management.