Hofland J, Feelders R A, Brabander T, Franssen G J H, de Herder W W
Department of Internal Medicine, ENETS Centre of Excellence, Erasmus Medical Centre, Rotterdam, the Netherlands.
Neth J Med. 2018 Apr;76(3):100-108.
Well-differentiated neuroendocrine tumours (NETs) of the digestive tract are being increasingly detected, which is partly explained by the increased use of endoscopic and cross-sectional imaging as well as improved recognition at histopathological evaluation. After the discovery of this relatively indolent type of epithelial malignancy over 100 years ago, their sporadic occurrence and divergent biological behaviour at multiple primary sites have hampered dedicated studies into NET pathogenesis and testing of drug efficacy in well-designed clinical trials. The last decade, however, has seen significant improvements in the NET field regarding our understanding of their pathophysiology. This has been substantiated by novel and exciting diagnostic and therapeutic options, including superior positron emission tomography imaging, treatment with unlabelled and radiolabelled somatostatin analogues and inhibitors of the mammalian target of rapamycin and vascular endothelial growth factor pathways. This review summarises contemporary studies within NET patients, which have enriched our clinical repertoire for this disease and have been instrumental in securing a remarkable improvement of overall survival within recent years.
消化道高分化神经内分泌肿瘤(NETs)的检出率日益增加,部分原因是内镜检查和断层成像技术的使用增多,以及组织病理学评估中识别能力的提高。在100多年前发现这种相对惰性的上皮性恶性肿瘤后,其散发性发生以及在多个原发部位的不同生物学行为,阻碍了在精心设计的临床试验中对NET发病机制进行专门研究以及对药物疗效进行测试。然而,在过去十年中,我们对NET病理生理学的理解在NET领域取得了显著进展。这已通过新颖且令人兴奋的诊断和治疗选择得到证实,包括先进的正电子发射断层扫描成像、使用未标记和放射性标记的生长抑素类似物治疗以及雷帕霉素靶蛋白和血管内皮生长因子途径抑制剂。本综述总结了NET患者的当代研究,这些研究丰富了我们针对该疾病的临床方法,并在近年来显著提高总体生存率方面发挥了重要作用。