原发性肝脏神经内分泌肿瘤的诊断和治疗进展

Advances in Diagnosis and Treatment of Primary Hepatic Neuroendocrine Neoplasms

摘要: 原发性肝脏神经内分泌肿瘤(Primary hepatic neuroendocrine neoplasms, PHNENs)特指原发于肝脏的一组能够分泌生物活性胺和多肽类激素的异质性肿瘤，临床罕见，仅占所有NENs的0.3%~4.0%。大部分PHNENs的临床症状和影像学表现缺乏特异性，早期诊断困难，与转移性NENs难以鉴别。手术是治疗的首选，也是唯一可以根治的方法。对于不可切除的PHNENs，肝脏局部治疗有射频和肝动脉栓塞，系统治疗方法主要包括生长抑素类似物的生物治疗，哺乳动物雷帕霉素靶蛋白和酪氨酸激酶抑制剂的靶向治疗，肽受体放射性核素疗法和化疗。目前尚无单独针对PHNENs的治疗指南，本文就PHNENs的诊断和治疗进展作一综述。

Abstract: Primary hepatic neuroendocrine neoplasms (PHNENs) are a group of heterogenous tumors which originates from the liver and can secrete bioactive amines and polypeptide hormones. PHNENs are rare and represent only 0.3%~4.0% of all NENs. Most of PHNENs have no special clinical symptoms and imaging findings. It is difficult to be early diagnosed and distinguished from metastatic NENs. Surgery is the first choice of treatment and the only cure. Radiofrequency and hepatic artery em-bolization are used in the local treatment of unresectable PHNENs. The systematic therapies for PHNENs include biotherapy with somatostatin analogues, targeted therapies with mammalian ra-pamycin target protein and tyrosine kinase inhibitors, peptide receptor radionuclide therapy and chemotherapy. There is no definite guideline for treatment of PHNENs. This article reviewed the advances in diagnosis and treatment of PHNENs.