Abstract: Objective Hepatic neuroendocrine neoplasm has a relatively low incidence and there are few relevant studies. This study analyzed and summarized the diagnosis and treatment of 35 cases of hepatic neuroendocrine neoplasms and discusses the pathogenesis, diagnosis and treatment in combination with relevant literatures. Methods Clinicopathological data of 35 hepatic neuroendocrine neoplasm patients treated in Sun Yat-sen Memorial Hospital of Sun Yat-sen University were analyzed retrospectively. Results According to the WHO classification standard of digestive system tumors in 2010, 3 cases with primary neuroendocrine tumor, 5 cases with primary neuroendocrine tumor, and 4 case with secondary neuroendocrine tumor G2 and 23 cases with secondary neuroendocrine carcinoma. Of 35 cases, 12 received hepatectomy, 3 received transcatheter arterial chemoembolization （TACE）, 15 received systemic chemotherapy, and 7 received radiofrequency ablation. Other treatment modalities involved anhydrous alcohol ablation, sunitinib and octreotide injection. All patients underwent long-term follow-up. By the date of the last follow-up, 28 patients died, 7 patients survived, and the median survival time of hepatic neuroendocrine neoplasm patients were 22 months. Conclusion Hepatic neuroendocrine neoplasm is a rare heterogeneous tumor, the diagnosis of which is mainly depended on histopathology and immunohistochemistry. Multidisciplinary comprehensive therapy can help prolong the survival time of patients.

Key words: hepatic neuroendocrine neoplasm, hepatectomy, chemotherapy, targeted therapy

摘要： 目的 肝脏神经内分泌肿瘤发病率较低,相关研究较少,本文通过对35例肝脏神经内分泌肿瘤的诊治过程进行分析总结,并结合相关文献探讨其发病情况及诊治要点。方法 回顾性分析2003-01-01至2019-08-27中山大学孙逸仙纪念医院诊治的35例肝脏神经内分泌肿瘤患者的临床资料。结果 根据2010版消化系统肿瘤WHO分类标准,分为原发性神经内分泌瘤3例,原发性神经内分泌癌5例,继发性神经内分泌瘤G2级4例,继发性神经内分泌癌23例。12例患者接受肝切除手术,3例患者接受肝动脉化疗栓塞治疗,15例接受全身化疗,7例患者接受射频消融术,其他治疗方法包括无水酒精消融、舒尼替尼或奥曲肽治疗等。所有患者均接受长期随访,截至末次随访日期,28例患者死亡,7例患者生存,中位生存时间22个月。结论 肝脏神经内分泌肿瘤是一类罕见的异质性肿瘤,病理组织学和免疫组化是确诊的主要手段,多学科综合治疗有助于延长患者的生存时间。

关键词: 肝脏神经内分泌肿瘤, 肝切除术, 化疗, 靶向治疗