Castle Jennifer T, Levy Brittany E, Chauhan Aman
Department of Surgery, Markey Cancer Center, University of Kentucky, 800 Rose Street, Lexington, KY 40536, USA.
Department of Surgery, University of Kentucky, 800 Rose Street, Lexington, KY 40536, USA.
Cancers (Basel). 2022 Oct 15;14(20):5049. doi: 10.3390/cancers14205049.
Neuroendocrine neoplasms (NENs) encompass a variety of neuroendocrine tumors (NETs) and neuroendocrine carcinomas (NECs) which can arise anywhere in the body. While relatively rare in the pediatric population, the incidence of NENs has increased in the past few decades. These neoplasms can be devastating if not diagnosed and treated early, however, symptoms are variable and can be indolent for many years. There is a reported median of 10 years from the appearance of the first symptoms to time of diagnosis. Considering some of these neoplasms have a mortality rate as high as 90%, it is crucial healthcare providers are aware of NENs and remain vigilant. With better provider education and easily accessible resources for information about these neoplasms, awareness can be improved leading to earlier disease recognition and diagnosis. This manuscript aims to provide an overview of both the most common NENs as well as the rarer NENs with high lethality in the pediatric population. This review provides up to date evidence and recommendations, encompassing recent changes in classification and advances in treatment modalities, including recently completed and ongoing clinical trials.
神经内分泌肿瘤（NENs）包括多种神经内分泌瘤（NETs）和神经内分泌癌（NECs），它们可发生于身体的任何部位。虽然NENs在儿科人群中相对罕见，但在过去几十年中其发病率有所上升。如果不及早诊断和治疗，这些肿瘤可能具有毁灭性，然而，症状各不相同，且可能多年不明显。据报道，从首次出现症状到确诊的中位时间为10年。鉴于其中一些肿瘤的死亡率高达90%，医疗保健提供者了解NENs并保持警惕至关重要。通过更好的提供者教育以及获取有关这些肿瘤的信息的便捷资源，可以提高认识，从而实现疾病的早期识别和诊断。本手稿旨在概述儿科人群中最常见的NENs以及具有高致死率的罕见NENs。本综述提供了最新的证据和建议，涵盖了分类的最新变化和治疗方式的进展，包括最近完成和正在进行的临床试验。
