儿童肝间叶性错构瘤:CT、病理及临床对照研究

Hepatic Mesenchymal Hamartoma in Children:A Comparative Study of CT,Pathology and Clinical Features

目的分析儿童肝间叶性错构瘤(HMH)的CT、病理及临床特征,以提高对HMH的诊断和治疗水平。方法回顾性分析经手术病理证实的13例儿童HMH的CT、病理和临床特点,并复习文献。结果肝右叶9例,肝尾状叶2例,跨叶2例;单发12例,多发1例;肿瘤呈多房囊性(5/13)、多房囊实性(5/13)、单囊性(1/13)、实性为主(2/13)4种形态;肿块体积为82.3~1117.4 ml,平均673.7 ml,肿瘤与肝脏体积比值为0.32~0.75;典型的CT表现为肝内巨大多房囊性或囊实性肿块,平扫囊壁、间隔和/或实性部分呈稍低密度,增强扫描囊壁、间隔和/或实性部分呈渐进性或持续性强化,动脉期可见多条小血管穿行;肿瘤镜下由不同比例间叶成分、胆管、淋巴管、血管、肝细胞岛(肝细胞索)及大小不一的囊腔构成,间叶组织疏松水肿。结论对于2岁以下的婴幼儿,当腹部无痛性肿块逐渐增大,CT表现为肝脏内边界清楚的囊性、囊实性或实性肿块,伴有血清AFP水平正常或轻度升高时,应考虑HMH的可能性。明确该病的影像学表现,并将其与病理结果建立相关性,有助于缩小鉴别诊断的范围,以确保及时诊断和有效降低病死率。

Objective Highlighting the main computed tomography(CT)manifestations of pediatric hepatic mesenchymal hamartoma(HMH)to enhance its diagnosis and treatment.Methods Retrospective analysis of 13 cases of pediatric HMH with surgical pathological confirmation,including their CT findings,pathological characteristics,clinical features,and a literature review.Results There were 9 cases in the right hepatic lobe,2 cases in the caudate lobe and 2 cases crossing multiple lobes.Among them,12 cases were solitary lesions,and 1 case had multiple lesions.The tumors exhibited four different morphological patterns,including multilocular cystic(5/13),multilocular cystic-solid(5/13),simple cystic(1/13),and solid(2/13).Typical CT manifestations included large hepatic multilocular cystic or cystic-solid masses.On non-contrast scans,the cystic walls,septa,and/or solid portions appeared slightly hypodense,while on contrast-enhanced scans,the cystic walls,septa,and/or solid components showed progressive or persistent enhancement.Microscopically,the tumor is composed of varying proportions of mesenchymal components,bile ducts,lymphatic vessels,blood vessels,hepatocyte cords,and cavities of different sizes,with loose and edematous mesenchymal tissue.Conclusion In infants and toddlers under 2 years of age,consider the possibility of HMH when a painless abdominal mass is progressively enlarging,with CT findings revealing a large,well-defined cystic,cystic-solid,or solid mass within the liver,accompanied by normal or slightly elevated serum AFP levels.To ensure timely diagnosis and effectively lower mortality rates,it is crucial to identify the disease's radiological presentations and establish correlations between these radiological features and pathological findings,which help narrow the scope of differential diagnosis.