a Department of Oncology , University Campus Biomedico of Rome , Rome , Italy.
b Oncology Department , Antwerp University Hospital , Edegem , Belgium.
Expert Rev Gastroenterol Hepatol. 2018 May;12(5):471-478. doi: 10.1080/17474124.2018.1463157. Epub 2018 Apr 18.
Pancreatic neuroendocrine tumors (pNETs) represent a small part of pancreatic neoplasms, and the knowledge about their indolent clinical course remains a subject of investigation. They occur sporadically or as part of familial cancer syndromes and are classified by WHO in 3 categories. There is ongoing research to understand their molecular profiling and leading mutations. Areas covered: The aim of this review is to clarify the overall aspects of tumorigenesis, to expose the latest developments in understanding the course of the disease and the possible therapeutic implications of these. The review also discusses functional and non-functional pNETs and associated inherited syndromes as well as pNET molecular profiling and its possible guidance in the use of targeted therapy. Expert commentary: In the next decade, a more extensive application of new technologies will help improve quality of life and survival, individualizing treatment protocols and identifying which therapeutic strategy is more suitable for each kind of NET.
胰腺神经内分泌肿瘤（pNETs）是胰腺肿瘤的一小部分，关于其惰性临床病程的知识仍然是研究的主题。它们是散发性的或作为家族性癌症综合征的一部分发生的，并被世界卫生组织（WHO）分为 3 类。目前正在进行研究以了解其分子特征和主要突变。
本综述的目的是阐明肿瘤发生的整体方面，揭示了解疾病过程的最新进展以及这些进展可能带来的治疗意义。该综述还讨论了功能性和非功能性 pNETs 以及相关的遗传综合征，以及 pNET 的分子特征及其在靶向治疗中的可能指导作用。
在未来十年，新技术的更广泛应用将有助于提高生活质量和生存率，使治疗方案个体化，并确定哪种治疗策略更适合每种 NET。
