婴儿型肝脏血管内皮细胞瘤影像学诊断

Imaging diagnosis of infantile hepatic hemangioendothelioma

目的总结婴儿型肝脏血管内皮细胞瘤的CT及MR表现。方法回顾性分析10例婴儿型肝脏血管内皮细胞瘤的影像学表现,所有病例均行CT平扫和增强扫描,6例行MR平扫和增强。结果 CT平扫表现为肝内多发或单发的边界清楚的低密度病灶,单发肿块病灶2例含斑片状钙化影;增强扫描早期多发病灶呈结节状明显强化,延迟扫描可见肿瘤由周边向中心递进强化。单发巨大病灶边缘强化为著,中心坏死区始终未见强化,CT随访6例病灶均不同程度缩小;6例MR平扫表现为肝内的长T1长T2占位信号,边界清晰,2例病灶周围可见增粗血管。结论婴儿型肝脏血管内皮细胞瘤表现为多发或单发,CT特征性表现为增强后呈结节状强化,巨大单发病灶中心坏死区强化不显著,MR病灶表现为稍长T1、长T2信号,增强后病灶明显强化,提示肝动脉供血;多发结节型激素治疗效果好,6个月随访提示病灶明显缩小或减少。

Objective To analyze and summarize the CT and MR findings of infantile hepatic hemangioendothelioma（IHHE）.Methods Imaging findings in 10 cases with IHHE were reviewed retrospectively analyzed.CT was performed in 10 patients and magnetic resonance imaging（MRI） was performed in 6 cases.Results At noncontrast CT,the IHHE manifested as single or multiple,well-defined and in hypodense masses.In larger tumors,central necrosis was with calcifications.At postcontrast CT,the enhancement patterns of IHHE include nodular,heterogeneous or peripheral enhancement in the early phase and subsequent central enhancement with variable delay scans.In larger tumors,central necrosis lacked enhancement in all phrases.The follow-up CT scans of 6 cases given steroids showed the masses shrinked strikingly or disappeared completely.According to the MRI findings of 6 cases,the multiple nodules showed hypointense on T1-weigehted inaging and hyperintense on T2-weighted imaging,sharpness of border,2 cases of vascular lesions seen around thickening thick.Conclusion The typical imaging features of HHE include two types： singe,giant mass with peripheral enhancement or multiple lesions with nodular enhancement.Given steroids,the IHHE has a natural history of regression and involution.Especially,the multiple lesions tend to disappear completely within six months.