论文部分内容阅读
目的原发性肝脏神经内分泌肿瘤(primary hepatic neuroendocrine neoplasm,PHNEN)是罕见肿瘤。本研究通过对13例PHNEN的诊治过程进行分析总结,并结合相关文献深入探讨诊治要点。方法回顾性分析2012-03-01-2014-07-31中国人民解放军第八一医院诊治的13例PHNEN患者临床资料。结果根据2010版消化系统肿瘤WHO分类标准,分为神经内分泌瘤(neuroendocrine tumor,NET)G1 1例,NET G2 6例,神经内分泌癌(neuroendocrine carcinoma,NEC)6例。根据AJCC第7版肝细胞癌分期标准,分为Ⅰ期1例,ⅢA期6例,ⅣA期2例,ⅣB期4例。2例患者接受肝脏姑息性切除手术,5例接受全身化疗,5例接受肝动脉化疗栓塞(transcatheter arterial chemoembolization,TACE)治疗,其他治疗方法包括放疗、舒尼替尼或奥曲肽微球等。所有患者均接受长期随访,截至末次随访日期,9例患者死亡,4例患者生存,中位生存时间12个月。1例接受TACE治疗的PHNET G1患者生存期为12个月;6例PHNET G2患者已有3例死亡,中位生存期12个月;6例PHNEC患者中有4例接受化疗,其中3例获得PR,6例PHNEC患者的中位生存期为12个月。结论 PHNEN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方法,综合治疗方法有助于延长患者的生存时间。
Objective Primary hepatic neuroendocrine neoplasm (PHNEN) is a rare tumor. In this study, 13 cases of PHNEN diagnosis and treatment process were analyzed and summarized, combined with the relevant literature in-depth discussion of the main points of diagnosis and treatment. Methods The clinical data of 13 PHNEN patients diagnosed and treated by the 81st Hospital of People ’s Liberation Army from 2012-03-01-2014-07-31 were retrospectively analyzed. Results According to WHO classification criteria of 2010 digestive system tumors, there were 1 case of neuroendocrine tumor (NET) G1, 6 cases of NET G2 and 6 cases of neuroendocrine carcinoma (NEC). According to the seventh edition of AJCC staging criteria of hepatocellular carcinoma, there were 1 stage in stage Ⅰ, 6 stage in stage ⅢA, 2 stage in stage ⅣA and 4 stage ⅣB. Two patients received palliative resection of the liver, five received systemic chemotherapy, five received transcatheter arterial chemoembolization (TACE), and other therapies included radiotherapy, sunitinib, or octreotide microspheres. All patients underwent long-term follow-up. As of the last follow-up date, 9 patients died and 4 patients survived with a median survival of 12 months. One patient with PHNET G1 who received TACE had a survival of 12 months; 3 of 6 patients with PHNET G2 had a median survival of 12 months; and 4 of 6 patients with PHNEC received chemotherapy, of which 3 received PR, 6 patients with PHNEC had a median survival of 12 months. Conclusion PHNEN is a rare heterogeneous tumor. Histopathology and immunohistochemistry are the main methods for diagnosis. PHNEN can prolong the survival time of patients.