原发性肝脏神经内分泌肿瘤临床特征分析

Analysis of clinical features of primary hepatic neuroendocrine neoplasms

目的探讨原发性肝脏神经内分泌肿瘤(primary hepatic neuroendocrine neoplasms,PHNENs)临床特点,以期优化PHNENs诊疗方法。方法收集南京医科大学第一附属医院2010年1月至2018年6月2例经病理诊断为PHNENs患者的临床资料,包括人口统计学资料、实验室检查、肿瘤的特征组织和病理学特点等。并通过检索万方及PubMed数据库,统计2010年1月至2018年6月关于PHNENs的26例个案报道中患者资料,进行回顾性分析。结果本研究共纳入28例PHNENs患者,发病中位年龄为51.5岁(6~87岁),其中女性17例(60.7%)。病理分级为G 1者2例(7.1%),均拒绝治疗,1例18个月死亡,1例出院1个月后失访;G 2者16例(57.1%),其中7例接受单纯手术切除治疗,4例接受肝动脉化疗栓塞(transhepatic arterial chemoembolization,TACE)治疗,2例行肝移植手术,2例接受多方案治疗,1例治疗方法不详;G 3者10例(35.7%),其中5例接受单纯手术治疗,1例接受放化疗,1例拒绝治疗,3例文献中未提及治疗方案。结论诊断PHNENs不仅取决于肝脏活检的病理学结果,还需要通过各项检查排除肝外肿瘤转移的可能性。手术治疗应作为首选的治疗方法,其余治疗包括化疗、TACE、射频消融、放疗。

Objective To investigate the clinical characteristics for optimizing the diagnosis and treatment of primary hepatic neuroendocrine neoplasms(PHNENs).Methods The clinical data of 2 patients diagnosed with PHNENs from the First Affiliated Hospital of Nanjing Medical University and 26 patients diagnosed with PHNENs from related literatures(WanFang database and PubMed)from Jan.2010 to Jun.2018 were collected.Demographic data,laboratory examination,characteristic and pathological features of tumors were recorded.Results There were 28 patients with PHNENs involved.The median age of them was 51.5 years old(ranged from 6 to 87 years old),and 17 patients(60.7%)were female.Among the 28 patients,2 cases(7.1%)were at G 1,both of which refused any therapy.16 patients(57.1%)were at G 2,of which 7 cases underwent surgical resection,4 cases underwent transhepatic arterial chemoembolization(TACE)treatment,2 cases underwent liver transplantation,2 cases were treated with various therapies,and 1 patient’s treatment was unknown.10 cases(35.7%)were at G 3,of which 5 cases underwent simple surgery,1 case underwent radiotherapy and chemotherapy,one case refused treatment,and 3 patients’therapies were unknown.Conclusion The diagnosis of PHNENs depends not only on the pathological results of liver biopsy,but also on the examinations to exclude the possibility of extrahepatic origins.Surgical resection should be used as the preferred treatment,and the remaining treatment including chemotherapy,TACE,radiofrequency ablation,radiotherapy.