4例原发性肝脏神经内分泌肿瘤临床分析及文献复习

Primary hepatic neuroendocrine neoplasm: An analysis of 4 cases and literature review

目的总结原发性肝脏神经内分泌肿瘤(PHNEN)患者的病例资料,分析其临床特征,以提高临床诊断和治疗水平。方法回顾性分析、总结我院诊治PHNEN患者的临床表现、化验检查、影像学特点、病理学检查和治疗转归。结果4例患者均为女性,中位年龄为56(40,73)岁;2例无不适主诉,2例有腹痛;血清ALT 28.3(18.0~49.8)U/L,TBIL 11.5(9.5~16.3)μmol/L,神经元特异性烯醇化酶(NSE)为44.1(20.2~77.1)ng/ml;腹部CT均提示肝内多发占位,增强扫描动脉期呈环形或不均匀强化,静脉期强化程度降弱;组织病理学检查示3例为神经内分泌癌,1例为神经内分泌瘤G2;1例患者接受对症治疗,采取手术、介入和/或药物等联合治疗另3例患者,随访11~81个月,2例死亡,2例生存。结论PHNEN临床少见,缺乏特征性临床表现、辅助检查和病理学诊断标志,需排除其他病变后方可考虑诊断。早期发现和联合治疗是改善患者预后的重要策略。

Objective The aim of this study was to summarize the clinical features of patients with primary hepatic neuroendocrine neoplasm(PHNEN).Methods The clinical manifestations,laboratory tests,imaging,pathological examination and clinical outcomes in four patients with PHNEN encountered in our hospital were summarized by retrospective analysis.Results The age of the four female patients was 56(40-73)years;there were no symptoms in 2 cases and abdominal pain in 2 cases;serum ALT levels were 28.3(18.0-49.8)U/L,bilirubin levels were 11.5(9.5-16.3)μmol/L,neuron-specific enolase levels were 44.1(20.2-77.1)ng/ml;the abdominal CT scan showed multiple lesions in all cases,and in contrast-enhanced scan,different degrees of ring or uneven enhancement in the arterial phases,and decreased enhancement in the venous phase;all diagnoses were confirmed by pathological examination,and surgical operation,interventional therapy and chemotherapy were given in 3 cases,and one case did not receive any treatment;during the follow-up period of 11 to 81 months,2 cases died and 2 cases still survived.Conclusion The PHNEN is a rare tumor in clinic,and lacks of specific laboratory and pathological markers.It should be considered after excluding other liver tumors.Early detection and several therapy combination are recommended to improve the prognosis.