274解放军医学院学报 Acad J Chin PLA Med Sch Mar 2016，37（3） http://jyjxxyxb.paperopen.com病例报告5 例原发性肝神经内分泌肿瘤的诊治杨 璞，刘家宏，周 旋，肖文华，朱建华解放军总医院第一附属医院 肿瘤一科，北京 100048摘要 ：目的 探讨原发性肝神经内分泌肿瘤的诊疗方法。方法 回顾性分析 2013 年 1 月 - 2014 年 7 月本院收治的 5 例原发性肝神经内分泌肿瘤的病例资料。结果 5 例均经病理确诊，且未发现肝外病灶。男性 3 例 (60%)，女性 2 例 (40%)，发病年龄 38 ~ 77 岁，平均年龄 61.2 岁。4 例确诊时即发现肝内转移，无手术机会。2 例行单纯肝动脉栓塞化疗，2 例化疗联合靶向治疗，1 例化疗联合肝动脉栓塞。行化疗的 3 例病例中 2 例联合应用生长抑素类似物。最终 2 例失访，1 例死亡，2 例存活至今。结论 原发性肝神经内分泌肿瘤早期诊断困难，可综合运用局部栓塞、化疗、靶向治疗及生长抑素类似物治疗，提高患者生存率。关键词 ：神经内分泌肿瘤 ；肝肿瘤 ；治疗中图分类号 ：R 735.7 文献标志码 ：B 文章编号 ：2095-5227(2016)03-0274-04 DOI ：10.3969/j.issn.2095-5227.2016.03.020网络出版时间 ：2015-12-08 10:12:03 网络出版地址 ：http://www.cnki.net/kcms/detail/11.3275.R.20151208.1012.004.htmlDiagnosis and treatment of patients with primary hepatic neuroendocrine tumor: An analysis of 5 casesYANG Pu, LIU Jiahong, ZHOU Xuan, XIAO Wenhua, ZHU JianhuaNO.1 Department of Oncology, the First Affiliate Hospital of Chinese PLA General Hospital, Beijing 100048, ChinaCorresponding author: ZHU Jianhua. Email: zjhua168@sohu.comAbstract: Objective To explore the diagnosis and treatment of primary hepatic neuroendocrine tumors (PHNET). Methods Clinical data about 5 PHNET patients from January 2013 to July 2014 in NO.1 department of oncology in the First Affiliate Hospital of Chinese PLA General Hospital were retrospective analyzed. Results Diagnosis of PHNET was confirmed immunohistochemically. No tumors were found out of liver. There were 3 males (60%) and 2 females (40%) with average age of 61.2 years (range from 38 to 77 years). Four patients were diagnosed with intrahepatic metastasis and lost the opportunity of operation. Of the 5 cases, 2 patients received transcatheter arterial chemoembolization (TACE), 2 cases were treated with chemotherapy combined targeted therapy, and 1 case with chemotherapy and TACE. Of the 3 cases with chemotherapy, 2 cases received Somatostatin Analogs (SSA) treatment. Two patients were lost to follow-up, one patient died and two survived to the present. Conclusion PHNET is extremely rare with difficulty in early diagnosis. Curative liver resection integrated with TACE, chemotherapy, targeted therapy and SSA can be used to improve patient survival rate.Keywords: neuroendocrine tumors; liver neoplasms; therapy 神经内分泌肿瘤 (neuroendocrine neoplasm，NEN)是一类来源于神经内分泌系统的肿瘤，临床上较为少见。发生于肝的神经内分泌肿瘤多为胃肠胰神经内分泌肿瘤转移而来，原发性肝神经内分泌肿瘤 (primary hepatic neuroendocrine tumor，PHNET)非：奔。本文回顾分析临床工作中收治的 5 例原发性肝神经内分泌肿瘤，探讨该病的诊疗经验，以提高对该病的认识。病例资料 病例 1：患者男性，77 岁。因进行性消瘦 1 年，乏力 1 个月，发现腹部包块 4 d 于 2013 年 1 月 9日入院。患者入院前 1 年体质量进行性下降约 7 kg，近 1 个月乏力明显，无厌油、纳差、恶心等不适。入院前 4 d 晨起发现右上腹一凸起包块，质较硬。既往有冠心病、糖尿病病史，无乙肝病史。查体 ：腹平坦，触诊柔软，肝右肋下 10 cm、剑突下 6 cm，质地硬，有轻压痛，无反跳痛及肌紧张，脾肋下未触及，莫菲征阴性。门诊腹部超声检查提示 ：肝大，肝内多发中强回声包块。入院诊断 ：肝占位待查，恶性可能性大。入院后查腹部 CT 检查提示 ：1) 肝内多发密度减低影，考虑恶性病变可能性大，肝癌并肝内多发转移或肝多发转移癌可能 ；2) 右肺下叶结节影，恶性病变待排。全身PET/CT 检查提示 ：肝多发低密度影，代谢明显增高，符合肝癌伴肝内转移 ；双肺多发结节影 ；头部及余躯干 PET/CT 检查未见明显异常。肝功能检查结果 ：碱性磷酸酶 146 U/L、谷氨酰转肽酶 299 收稿日期 ：2015-09-06作者简介 ：杨璞，女，在读硕士。研究方向 ：常见肿瘤的诊治。Email: oppoyang_07@sina.com通信作者 ：朱建华，女，博士，副主任医师。Email: zjhua168@sohu.com
