原发肝脏神经内分泌肿瘤的临床及影像特征

Clinical characteristics and imaging features of primary hepatic neuroendocrine neoplasm

目的探讨原发肝脏神经内分泌肿瘤(primary hepatic neuroendocrine neoplasms,PHNENs)的临床及影像学特点。材料与方法回顾性分析我院2011年1月至2019年1月经病理确诊的11例PHNENs的临床及影像学资料。结果1例有肝病史,余均无肝病史。神经元特异性烯醇化酶(neuron-specific enolase,NSE)的阳性率为75%。11例患者的病理分型为神经内分泌瘤(neuroendocrine tumors,NET)6例(G1=1;G2=5),神经内分泌癌(neuroendocrine carcinoma,NEC)5例。PHNENs中单发病灶5例(NET=4;NEC=1),多发6例(NET=2;NEC=4),其中4例为大灶旁伴子灶;7例病灶密度/信号不均(NET=3;NEC=4),其中含囊变4例,出血2例,钙化1例;2例病灶含液-液平;病灶在MR扩散加权成像上均显示为受限的高信号(n=6)。增强扫描动脉期9例表现为实性显著强化,8例快进快出,其中4例行MR肝胆期扫描后均为低信号。数字减影血管造影示肿瘤均呈富血供染色。结论PHNENs的临床及影像表现具有一定的特征,为诊断提供依据。

Objective:To investigate the clinical characteristics and imaging features of primary hepatic neuroendocrine neoplasms(PHNENs).Materials and Methods:Clinical pathological information and imaging features of 11 histopathologically confirmed PHNEN patients were retrospectively reviewed.Results:All the patients were negative for liver hepatitis cirrhosis except one.The positive rate for neuron-specific enolase(NSE)was 75%.The histopathologic classifications of the 11 cases were NET(n=6;G1=1,G2=5)and NEC(n=5).The PHNENs can be single lesion(NET=4;NEC=1)or multiple lesions(NET=2;NEC=4).Seven cases(NET=3;NEC=4)were heterogeneous for the cystic change(n=4),hemorrhage(n=2)and calcification(n=1).Two patients had fluid-fluid levels.The lesions were restricted on MR diffusion weighted image(DWI)(n=6).Nine cases showed strong global enhancement and 8 cases exhibited wash out including 4 patients with hepatobiliary phase images showing hypointensity.Tumors on digital subtraction angiography(DSA)were manifested as hypervascular mass.Conclusions:PHNENs has specific features on clinics and imaging findings and they are valuable in the diagnosis and differential diagnosis.