胃肠道神经内分泌肿瘤的诊疗进展

Progress of Diagnosis and Treatment of Gastrointestinal Neuroendocrine Tumors

目的总结胃肠道神经内分泌肿瘤(GE-NETs)的病理学分类、临床表现、诊断与治疗。方法通过查阅国内外相关文献,对GE-NETs的临床研究现状与治疗方法进行综述。结果 GE-NETs起源于具有胺前体摄取和脱羧作用的肠嗜铬细胞,其进展缓慢,能产生多种肽类激素,从而引起特征性的内分泌综合征。目前,在其病理学诊断、肿瘤分级、TNM分期及预后判断方面尚有一定分歧。该病的早期诊断率偏低,通常在诊断时已经发生转移,肝脏是常见的转移部位。影像学检查在其早期诊断中占据重要地位。手术是其一线治疗方案,且是唯一可达到治愈目的的有效治疗方法。生长抑素类似物如长效奥曲肽能缓解肿瘤所导致的临床症状并延缓其进展,且与生物靶向制剂如依维莫司联合应用表现出协同抑制肿瘤增殖的作用。结论关于GE-NETs诊断、分级等方面的分歧成为治疗GE-NETs的阻碍因素,个体化综合治疗是未来的目标。

Objective To summary the pathological classification,clinical manifestations,diagnosis,and treatments of gastrointestinal neuroendocrine tumors（GE-NETs）.Methods Domestic and international literatures were collected to summary the status of clinical researches and treatments of GE-NETs.Results GE-NETs derived from enterochromaffin cells throughout the gut which had the function of amine precursor uptake decarboxylase（APUD）.These tumors secreted discrete bioactive substances and produced characteristic immunohistochemical patterns,making patients to manifest endocrine syndrome.But there were no unified standards on the diagnosis,grade,TNM classification,and prognosis of GE-NETs.Early diagnosis rate of GE-NETs was low,and most of tumors were asymptomatic and detected at late stage,with a tendency to metastasize to the liver.Imaging examation was important in early diagnosis of GE-NETs.Surgery was the traditional first-line therapy and the only possible curative approach.Somatostatin analogues,such as long-term-release（LAR） octreotide,could relieve the hormonal symptoms,slow down the tumor growth,and had shown synergistic antiproliferative activity in combination with biological agent everolimus.Conclusions The lack of standardized classification and an incomplete understanding of this disease are some of the impediments to the progress of treatment.Individualized comprehensive therapy is the aim of the future treatment.