Well differentiated neuroendocrine tumors (NETs) arising in the gastrointestinal and pancreaticobiliary system are the most common neuroendocrine neoplasms. Studies of the molecular basis of these lesions have identified genetic mutations that predispose to familial endocrine neoplasia syndromes and occur both as germline events and in sporadic tumors. The mutations often involve epigenetic regulators rather than the oncogenes and tumor suppressors that are affected in other malignancies. Somatic copy number alterations and miRNAs have also been implicated in the development and progression of some of these tumors. The molecular profiles differ by location, but many are shared by tumors in other sites, including those outside the gastroenteropancreatic system. The approach to therapy relies on both the neuroendocrine nature of these tumors and the identification of specific alterations that can serve as targets for precision oncologic approaches.

起源于胃肠道和胰胆系统的分化良好的神经内分泌肿瘤（NETs）是最常见的神经内分泌肿瘤。对这些病变的分子基础的研究已经确定了易患家族性内分泌肿瘤综合征的基因突变，这些突变既作为种系事件发生，也发生在散发性肿瘤中。这些突变通常涉及表观遗传调节因子，而不是其他恶性肿瘤中受影响的癌基因和肿瘤抑制因子。体细胞拷贝数改变和 miRNA 也与其中一些肿瘤的发展和进展有关。分子谱因位置而异，但许多是由其他部位的肿瘤共享的，包括胃肠胰腺系统以外的肿瘤。