摘要
目的针对临床罕见的原发性肝脏神经内分泌肿瘤(PHNENs)的临床表现、病理学特点、治疗方法及易误诊的特性进行探讨。方法回顾性分析13例初诊为PHNENs患者的临床表现、病理、治疗方法以及随访结果,对其分析并对相关文献进行复习。结果PHNENs多数无症状或仅有腹痛等非特异临床表现,影像表现很难与其他肝脏恶性肿瘤鉴别;病理及免疫组化结果是主要诊断依据,长期随访发现部分为胃肠胰神经内分泌肿瘤的肝转移。结论PHNENs是一种极罕见的原发于肝脏的神经内分泌肿瘤,确诊需要长期的随访支持,临床常见PHNENs不能除外转移性神经内分泌肿瘤。治疗是以手术为主,肝动脉化疗栓塞、射频消融、生长抑素类似物、化疗、靶向等综合治疗可能延长生存期。
Objective:To investigate the clinical manifestations,pathological features,treatment methods and misdiagnosis of a kind of rare liver tumor,primary hepatic neuroendocrine neoplasms(PHNENs).Methods:The clinical manifestations,pathology,treatment methods and follow-up results of 13 initially diagnosed PHNENs patients were analyzed retrospectively,and the relevant literatures were reviewed.Results:Most of PHNENs had no symptoms or only non-specific clinical manifestations such as abdominal pain,and the imaging manifestations were difficult to differentiate from other liver malignant tumors.Pathological and immunohistochemical results were the main diagnostic evidence.A small number of primary hepatic neuroendocrine tumors were found to be hepatic metastases of neuroendocrine tumors from the gastrointestinal and pancreatobiliary tracts through long-term follow-up.Conclusions:PHNENs is a kind of rare primary neuroendocrine tumor in the liver,which needs long-term follow-up to exclude metastatic of neuroendocrine tumors.Hepatectomy is the main treatment of PHNENs.The combined therapy of transcatheter arterial chemoembolization(TACE),radiofrequency ablation,somatostatin analogue therapy,chemotherapy and targeted therapy may prolong the survival period.
作者
刘哲
李晓明
蔡守旺
孔哲
辛宪磊
刘志伟
Liu Zhe;Li Xiaoming;Cai Shouwang;Kong Zhe;Xin Xianlei;Liu Zhiwei(Department of Hepatobiliary Surgery,First Center of General Hospital PLA,Beijing 100853,China;Department of Hepatobiliary Surgery,Shandong Provincial Otorhinolaryngology Hospital Affiliated to Shandong University,Ji'nan 250023,China)
出处
《中华转移性肿瘤杂志》
2020年第1期52-56,共5页
Chinese Journal of Metastatic Cancer
关键词
肝脏肿瘤
神经内分泌肿瘤
原发性肿瘤
误诊分析
Liver tumor
Neuroendocrine neoplasms
Primary neoplasms
Misdiagnosis analysis
