Hofland Johannes, de Herder Wouter W.
Endocrinologist, Department of Internal Medicine, Division of Endocrinology, Erasmus MC & Erasmus MC Cancer Institute Rotterdam, the Netherlands
Professor of endocrine oncology, Department of Internal Medicine, Division of Endocrinology, Erasmus MC & Erasmus MC Cancer Institute, Rotterdam, the Netherlands
Neuroendocrine neoplasms originating from the gut are increasingly diagnosed as a result of the rise in radiological and endoscopic procedures, improved pathological classification, and likely an increase in true incidence. The diffuse neuroendocrine gastrointestinal system can trigger cancer formation into a wide variety of neoplasm subtypes, ranging from well-differentiated tumors to poorly differentiated carcinomas. All gastrointestinal neuroendocrine neoplasms have the potential to metastasize and ultimately impair patient survival. In recent years, changes have occurred in the pathophysiological understanding, nomenclature, pathological grading, molecular imaging, and management options for these neuroendocrine neoplasms. This chapter will focus on well-differentiated neuroendocrine tumors of gastrointestinal origin, which find their origin at separate primary locations, all characterized by their specific clinical behavior. A minority of patients suffer from hormonal syndromes due to the secretion of peptides or amines from the neuroendocrine tumor. The carcinoid syndrome is the quintessential hormonal syndrome in gastrointestinal neuroendocrine tumors, particularly those of midgut origin. Patients suffering from the carcinoid syndrome have a reduced survival and quality of life, due to debilitating symptoms of flushing and diarrhea as well as fibrotic complications. We provide an overview of the background of gastrointestinal neuroendocrine tumors as well as the carcinoid syndrome and discuss the diagnostic pathways as well as treatment possibilities for patients presenting with this disease. For complete coverage of all related areas of Endocrinology, please visit our on-line FREE web-text, WWW.ENDOTEXT.ORG.
由于放射学和内镜检查程序的增加、病理分类的改进以及实际发病率可能上升，源自肠道的神经内分泌肿瘤的诊断越来越多。弥漫性神经内分泌胃肠系统可引发癌症形成多种肿瘤亚型，从高分化肿瘤到低分化癌。所有胃肠神经内分泌肿瘤都有转移的可能，并最终影响患者生存。近年来，对这些神经内分泌肿瘤的病理生理学理解、命名、病理分级、分子成像和治疗选择都发生了变化。本章将重点关注源自胃肠道的高分化神经内分泌肿瘤，它们起源于不同的原发部位，都具有特定的临床行为特征。少数患者因神经内分泌肿瘤分泌肽或胺而出现激素综合征。类癌综合征是胃肠神经内分泌肿瘤，特别是中肠起源肿瘤的典型激素综合征。患有类癌综合征的患者由于潮红、腹泻等使人衰弱的症状以及纤维化并发症，生存和生活质量下降。我们概述了胃肠神经内分泌肿瘤以及类癌综合征的背景，并讨论了患有这种疾病的患者的诊断途径和治疗可能性。欲全面涵盖内分泌学的所有相关领域，请访问我们的在线免费网络文本，WWW.ENDOTEXT.ORG。
