神经内分泌肿瘤(NENs)可在几乎任何器官内发展，并跨越从良好分化和惰性神经内分泌肿瘤(NET)到恶性程度较高的神经内分泌癌(NEC)，包括小细胞肺癌(SCLC)的谱系。这些肿瘤被认为主要来源于遍布全身的神经内分泌前体细胞，也可通过器官特异性上皮细胞类型的神经内分泌转分化而产生。因此，NENs构成了一组肿瘤类型，无论其起源的部位如何，都具有相同的基因组和表型特征，尽管存在一些器官特异性差异。建立几个这些疾病实体的代表性临床前模型，以及对人类肿瘤标本的分析，为其生物学的重要方面提供了重要见解，这对治疗具有重要意义。在本综述中，我们从临床和生物学的角度，提供了对胃肠系统和肺NENs当前的理解全面概述。研究NENs通常被分隔开来到肿瘤起源的部位，而跨领域的视角可能能够促进研究中的进展，从而加速其他领域的研究。因此，我们的目标是强调对于不同器官中出现的NENs的共性和差异的更好理解，可能更有效地为临床研究界定治疗靶点，并最终优化患者护理。 © 2022年 Springer Nature有限公司。

Neuroendocrine neoplasms (NENs) can develop in almost any organ and span a spectrum from well-differentiated and indolent neuroendocrine tumours (NETs) to poorly differentiated and highly aggressive neuroendocrine carcinomas (NECs), including small-cell lung cancer (SCLC). These neoplasms are thought to primarily derive from neuroendocrine precursor cells located throughout the body and can also arise through neuroendocrine transdifferentiation of organ-specific epithelial cell types. Hence, NENs constitute a group of tumour types that share key genomic and phenotypic characteristics irrespective of their site of origin, albeit with some organ-specific differences. The establishment of representative preclinical models for several of these disease entities together with analyses of human tumour specimens has provided important insights into crucial aspects of their biology with therapeutic implications. In this Review, we provide a comprehensive overview of the current understanding of NENs of the gastrointestinal system and lung from clinical and biological perspectives. Research on NENs has typically been siloed by the tumour site of origin, and a cross-cutting view might enable advances in one area to accelerate research in others. Therefore, we aim to emphasize that a better understanding of the commonalities and differences of NENs arising in different organs might more effectively inform clinical research to define therapeutic targets and ultimately optimize patient care.© 2022. Springer Nature Limited.